1.1.1.1 alcohol dehydrogenase mitochondrion - 5739 721749 1.1.1.1 alcohol dehydrogenase mitochondrion higher enzyme level in mitochondria from cells grown at pH 6.0 than in mitochondria from cells grown at pH 3.7 5739 739328 1.1.1.1 alcohol dehydrogenase mitochondrion HpADH3 5739 -, 721388 1.1.1.1 alcohol dehydrogenase mitochondrion KlDH3 and KlADH4 are genes encoding mitochondrial alcohol dehydrogenase activities located within the mitochondria. Ethanol induces the transcription of KlADH4 and, conversely, represses that of KlADH3 5739 668675 1.1.1.1 alcohol dehydrogenase mitochondrion KmAdh3 possesses an amino-terminal extension as a mitochondrial targeting sequence 5739 -, 685652 1.1.1.1 alcohol dehydrogenase mitochondrion KmAdh4 possesses an amino-terminal extension as a mitochondrial targeting sequence 5739 -, 685652 1.1.1.100 3-oxoacyl-[acyl-carrier-protein] reductase mitochondrion - 5739 704721, 740558 1.1.1.103 L-threonine 3-dehydrogenase mitochondrion - 5739 737579, 738933 1.1.1.105 all-trans-retinol dehydrogenase (NAD+) mitochondrion - 5739 656260 1.1.1.145 3beta-hydroxy-DELTA5-steroid dehydrogenase mitochondrion - 5739 389385, 389391, 389399, 389401, 740914, 762899, 763756 1.1.1.145 3beta-hydroxy-DELTA5-steroid dehydrogenase mitochondrion inner mitochondrial membrane 5739 389399 1.1.1.145 3beta-hydroxy-DELTA5-steroid dehydrogenase mitochondrion isoform 3betaHSD2 associates with the inner mitochondrial membrane or outer mitochondrial membrane translocases facing the intermembrane space. Tthis interaction promotes the conformational change needed for full activity. 3betaHSD2 associates with the inner mitochondrial membrane but does not integrate into the membrane. 3betaHSD2 forms a transient association with the translocases Tim50 and Tom22 and with Tim23. This association occurs primarily through the interaction of Tim50 with the N-terminus of 3betaHSD2 and contributes to enzymatic activity. Tim50 knockdown inhibits catalysis of dehydroepiandrostenedione to androstenedione and pregnenolone to progesterone. Although Tim50 knockdown decreases 3betaHSD2 expression, restoration of expression via proteasome and protease inhibition does not rescue activity 5739 725447 1.1.1.150 21-hydroxysteroid dehydrogenase (NAD+) mitochondrion isoform RDH13 5739 688677 1.1.1.178 3-hydroxy-2-methylbutyryl-CoA dehydrogenase mitochondrion - 5739 673530, 711851 1.1.1.2 alcohol dehydrogenase (NADP+) mitochondrion - 5739 286177, 286204, 286214, 654908 1.1.1.211 long-chain-3-hydroxyacyl-CoA dehydrogenase mitochondrion - 5739 -, 288681, 686162, 688119, 723341, 740102, 740106, 740438, 740557, 740641, 741457 1.1.1.237 hydroxyphenylpyruvate reductase mitochondrion - 5739 657023 1.1.1.27 L-lactate dehydrogenase mitochondrion - 5739 689715, 762363 1.1.1.27 L-lactate dehydrogenase mitochondrion inner compartment, transport and metabolism of L-lactate, overview 5739 685322 1.1.1.27 L-lactate dehydrogenase mitochondrion L-lactate dehydrogenase is restricted to the inner mitochondrial compartments 5739 747163 1.1.1.27 L-lactate dehydrogenase mitochondrion LDH is present in the inner mitochondrial compartments 5739 686648 1.1.1.27 L-lactate dehydrogenase mitochondrion LDH-A 5739 -, 685502 1.1.1.27 L-lactate dehydrogenase mitochondrion mL-LDH is restricted in inner compartment 5739 746989 1.1.1.277 3beta-hydroxy-5beta-steroid dehydrogenase mitochondrion - 5739 -, 389563 1.1.1.28 D-lactate dehydrogenase mitochondrion - 5739 -, 760993 1.1.1.28 D-lactate dehydrogenase mitochondrion D-lactate dehydrogenase is inserted into the inner membrane of mitochondria through its transmembrane domain located close to the N-terminus of the polypeptide chain in such a way that the protein globule is exposed in the intermembrane space 5739 667751 1.1.1.284 S-(hydroxymethyl)glutathione dehydrogenase mitochondrion - 5739 -, 724453, 762615, 762616, 762890, 763054 1.1.1.284 S-(hydroxymethyl)glutathione dehydrogenase mitochondrion presence of S-nitrosoglutathione, S-nitrosoglutathione reductase and nitrated proteins 5739 723896 1.1.1.284 S-(hydroxymethyl)glutathione dehydrogenase mitochondrion the enzyme contains a mitochondrial targeting peptide 5739 763605 1.1.1.30 3-hydroxybutyrate dehydrogenase mitochondrion - 5739 286514, 286515, 286516, 286517, 286518, 286519, 286521, 286522, 286525, 286527, 286528, 286529, 286530, 286535, 286542, 655126, 655261, 689351 1.1.1.30 3-hydroxybutyrate dehydrogenase mitochondrion beta-hydroxybutyrate dehydrogenase from heavy and light mitochondria are isoforms 5739 668322 1.1.1.30 3-hydroxybutyrate dehydrogenase mitochondrion light and heavy mitochondria, enzyme expression and activity is increased in heavy mitochondria in hibernating state, overview 5739 685409 1.1.1.30 3-hydroxybutyrate dehydrogenase mitochondrion located on the matrix face of the inner membrane 5739 286512, 286513, 286533, 286538 1.1.1.300 NADP-retinol dehydrogenase mitochondrion isoform RDH13 5739 688677 1.1.1.31 3-hydroxyisobutyrate dehydrogenase mitochondrion - 5739 688515, 722472 1.1.1.34 hydroxymethylglutaryl-CoA reductase (NADPH) mitochondrion - 5739 286557, 286560, 286564 1.1.1.34 hydroxymethylglutaryl-CoA reductase (NADPH) mitochondrion isozyme Hmg1 5739 686736 1.1.1.35 3-hydroxyacyl-CoA dehydrogenase mitochondrion - 5739 -, 286269, 286588, 286589, 286590, 286591, 286601, 286606, 286607, 286612, 33713, 657049, 657276, 673340, 673530, 684902, 685573, 687573, 690046, 723558 1.1.1.35 3-hydroxyacyl-CoA dehydrogenase mitochondrion matrix enzyme 5739 286603 1.1.1.36 acetoacetyl-CoA reductase mitochondrion isozyme AKR1B15.1 5739 740743 1.1.1.37 malate dehydrogenase mitochondrion - 5739 -, 286626, 286627, 286628, 286629, 286630, 286632, 286636, 286637, 286638, 286640, 286641, 286644, 286645, 286646, 286647, 286649, 286652, 286654, 286655, 286656, 286661, 286662, 286663, 286664, 286665, 286669, 286672, 286676, 655486, 656579, 656753, 656883, 656929, 669002, 670193, 679243, 689325, 696067, 700068, 700338, 700824, 721236, 740165, 760668, 760678 1.1.1.37 malate dehydrogenase mitochondrion highly expressed mMDH1 and lower expressed mMDH2 isoforms 5739 713318 1.1.1.37 malate dehydrogenase mitochondrion inner or outer membrane, m-MDH located in the inner membrane matrix 5739 286629 1.1.1.37 malate dehydrogenase mitochondrion isozyme mMDH 5739 711909, 712850 1.1.1.37 malate dehydrogenase mitochondrion Mdh1, matrix 5739 686953 1.1.1.37 malate dehydrogenase mitochondrion Mdh2 5739 723540 1.1.1.37 malate dehydrogenase mitochondrion mitochondrial isozyme 5739 688101 1.1.1.37 malate dehydrogenase mitochondrion mitochondrial MDH 5739 -, 711519 1.1.1.37 malate dehydrogenase mitochondrion outer membrane, intermembrane space, inner membrane, matrix 5739 -, 286653, 286672 1.1.1.38 malate dehydrogenase (oxaloacetate-decarboxylating) mitochondrion - 5739 286678, 286681, 286691, 286693, 286698, 656983, 685215, 695884, 741010, 760840 1.1.1.39 malate dehydrogenase (decarboxylating) mitochondrion - 5739 286704, 286708, 286715, 654651, 654720, 656163, 656983, 657321, 657362, 685215, 685622, 689604, 712417, 723540, 738272, 741121, 761183, 761186, 762109 1.1.1.39 malate dehydrogenase (decarboxylating) mitochondrion 65% of the activity 5739 286712 1.1.1.39 malate dehydrogenase (decarboxylating) mitochondrion isozyme ME2 5739 687568 1.1.1.39 malate dehydrogenase (decarboxylating) mitochondrion isozyme NAD-ME1 5739 711147 1.1.1.39 malate dehydrogenase (decarboxylating) mitochondrion isozymes ME2 and ME3 5739 712942 1.1.1.39 malate dehydrogenase (decarboxylating) mitochondrion isozymes NAD-ME1 and NAD-ME2 5739 711147 1.1.1.39 malate dehydrogenase (decarboxylating) mitochondrion ME2 5739 713373 1.1.1.39 malate dehydrogenase (decarboxylating) mitochondrion one of NAD-ME isoforms with MW 110 kDa is located in mitochondria of bundle sheath cells of control and drought-exposed plants, and another isoform of MW 121 kDa is formed in mitochondria of bundle sheath cells under influence of drought. After resuming watering the 121 kDa isoform disappears again. About 90.6% of the total NAD-ME activity is localized in mitochondrial stroma of bundle sheath cells 5739 741191 1.1.1.39 malate dehydrogenase (decarboxylating) mitochondrion predominantly in the matrix space 5739 286712 1.1.1.40 malate dehydrogenase (oxaloacetate-decarboxylating) (NADP+) mitochondrion - 5739 286715, 286734, 286735, 286738, 728207, 739163, 741010, 761074, 761183, 761332 1.1.1.40 malate dehydrogenase (oxaloacetate-decarboxylating) (NADP+) mitochondrion 2 distinct enzyme forms are present in cytosol and in mitochondria 5739 286725 1.1.1.40 malate dehydrogenase (oxaloacetate-decarboxylating) (NADP+) mitochondrion both cytosolic and mitochondrial isozymes are more abundant in the insect stage, although they can be immunodetected in the bloodstream forms 5739 -, 712042 1.1.1.40 malate dehydrogenase (oxaloacetate-decarboxylating) (NADP+) mitochondrion expression of the mitochondrial ME is upregulated in amastigotes 5739 -, 712042 1.1.1.40 malate dehydrogenase (oxaloacetate-decarboxylating) (NADP+) mitochondrion isozyme ME3 5739 687568 1.1.1.40 malate dehydrogenase (oxaloacetate-decarboxylating) (NADP+) mitochondrion mitochondrial isozyme, appears to be clearly more abundant in the insect stage, although it can be immunodetected in the bloodstream form 5739 -, 712042 1.1.1.40 malate dehydrogenase (oxaloacetate-decarboxylating) (NADP+) mitochondrion mitochondrial isozyme, the expression of the mitochondrial isozyme seems to be clearly upregulated in amastigotes 5739 -, 712042 1.1.1.40 malate dehydrogenase (oxaloacetate-decarboxylating) (NADP+) mitochondrion mitochondrial malic enzyme ME3 5739 712942 1.1.1.41 isocitrate dehydrogenase (NAD+) mitochondrion - 5739 -, 286749, 286754, 286761, 286762, 286766, 286767, 286774, 654652, 654843, 656118, 657111, 667647, 670544, 670547, 684713, 685719, 712440, 713076, 721381, 740114, 741407, 741458, 760834, 762407 1.1.1.41 isocitrate dehydrogenase (NAD+) mitochondrion exclusively 5739 654914 1.1.1.41 isocitrate dehydrogenase (NAD+) mitochondrion resticted to 5739 740425 1.1.1.42 isocitrate dehydrogenase (NADP+) mitochondrion - 5739 -, 286782, 286783, 286785, 286792, 286793, 654341, 654649, 654652, 655456, 655663, 656093, 656180, 668697, 669307, 669373, 670236, 670334, 686864, 686899, 689917, 696487, 711119, 739984, 740117, 740583, 760832, 761047, 762409, 762446 1.1.1.42 isocitrate dehydrogenase (NADP+) mitochondrion IDH2 5739 712614, 713508 1.1.1.42 isocitrate dehydrogenase (NADP+) mitochondrion IDH2 and IDH3 5739 713508 1.1.1.42 isocitrate dehydrogenase (NADP+) mitochondrion IDP1 5739 -, 711006 1.1.1.42 isocitrate dehydrogenase (NADP+) mitochondrion IDPm 5739 712077 1.1.1.42 isocitrate dehydrogenase (NADP+) mitochondrion increase in enzyme activity during ischemia, enzyme from ischemic heart mitochondria demonstrates higher activation energy and lower thermal stability and differs in KM-value and regulation 5739 670236 1.1.1.42 isocitrate dehydrogenase (NADP+) mitochondrion isoenzyme IDP1 5739 684709 1.1.1.42 isocitrate dehydrogenase (NADP+) mitochondrion isozyme ICD1, mainly 5739 656431 1.1.1.42 isocitrate dehydrogenase (NADP+) mitochondrion isozyme IDP1 5739 654367, 656233 1.1.1.42 isocitrate dehydrogenase (NADP+) mitochondrion isozyme mICDH 5739 656588 1.1.1.42 isocitrate dehydrogenase (NADP+) mitochondrion light-inducible isozyme 5739 654843 1.1.1.42 isocitrate dehydrogenase (NADP+) mitochondrion mitochondrial NADP+-dependent isocitrate dehydrogenase 5739 711330 1.1.1.42 isocitrate dehydrogenase (NADP+) mitochondrion mitochondrial targeting peptide signal is predicted for IDH1 5739 723129 1.1.1.49 glucose-6-phosphate dehydrogenase (NADP+) mitochondrion - 5739 287017 1.1.1.51 3(or 17)beta-hydroxysteroid dehydrogenase mitochondrion - 5739 287077, 677079 1.1.1.59 3-hydroxypropionate dehydrogenase mitochondrion - 5739 738640 1.1.1.62 17beta-estradiol 17-dehydrogenase mitochondrion - 5739 760700, 761037 1.1.1.62 17beta-estradiol 17-dehydrogenase mitochondrion isozyme 17beta-HSD10 5739 675983 1.1.1.62 17beta-estradiol 17-dehydrogenase mitochondrion isozyme AKR1B15.1 5739 740743 1.1.1.75 (R)-aminopropanol dehydrogenase mitochondrion - 5739 287300, 287301, 287307 1.1.1.79 glyoxylate reductase (NADP+) mitochondrion - 5739 286409, 287327, 761190 1.1.1.79 glyoxylate reductase (NADP+) mitochondrion intermembrane space and inner membrane 5739 287321 1.1.1.79 glyoxylate reductase (NADP+) mitochondrion isoform GLYR2 5739 761177 1.1.1.8 glycerol-3-phosphate dehydrogenase (NAD+) mitochondrion - 5739 667415, 712122 1.1.1.8 glycerol-3-phosphate dehydrogenase (NAD+) mitochondrion determined by isopycnic gradient centrifugation 5739 287358 1.1.1.8 glycerol-3-phosphate dehydrogenase (NAD+) mitochondrion isoform Gpd2p, contains mitochondrial presequence sufficient for targeting 5739 656246 1.1.1.8 glycerol-3-phosphate dehydrogenase (NAD+) mitochondrion localisation shown in Saccharomyces cerevisiae via GFP-fusion proteins 5739 675870 1.1.1.8 glycerol-3-phosphate dehydrogenase (NAD+) mitochondrion predicted from sequence 5739 675870 1.1.1.8 glycerol-3-phosphate dehydrogenase (NAD+) mitochondrion sn-glycerol 3-phosphate dehydrogenase generates superoxide about equally to each side of the membrane 5739 667785 1.1.1.80 isopropanol dehydrogenase (NADP+) mitochondrion - 5739 287362 1.1.1.82 malate dehydrogenase (NADP+) mitochondrion in matrix and intermembrane space and slightly in inner membrane 5739 286653 1.1.1.86 ketol-acid reductoisomerase (NADP+) mitochondrion - 5739 33969, 684636 1.1.1.B40 11beta-hydroxysteroid dehydrogenase (NAD+) mitochondrion isozyme 11beta-HSD2 5739 667203 1.1.2.3 L-lactate dehydrogenase (cytochrome) mitochondrion - 5739 -, 696507, 712523, 743847 1.1.2.3 L-lactate dehydrogenase (cytochrome) mitochondrion intermembrane space 5739 696302 1.1.2.3 L-lactate dehydrogenase (cytochrome) mitochondrion mitochondrial intermembrane space 5739 712759 1.1.2.4 D-lactate dehydrogenase (cytochrome) mitochondrion - 5739 -, 287801, 389623, 389624, 389625, 389631, 389633, 685358, 706465, 742052, 763102 1.1.2.4 D-lactate dehydrogenase (cytochrome) mitochondrion either on the inner side of the inner mitochondrial membrane or in the matrix 5739 672323 1.1.3.15 (S)-2-hydroxy-acid oxidase mitochondrion - 5739 389700, 389701, 741109 1.1.3.15 (S)-2-hydroxy-acid oxidase mitochondrion preparation of heavy and light mitochondria, 2 distinct molecular forms 5739 389693 1.1.3.17 choline oxidase mitochondrion membrane-bound 5739 287801 1.1.3.2 L-lactate oxidase mitochondrion intermembrane space. H2O2 is produced independently on the respiratory chain with 1:1 stoichiometry with pyruvate, due to a putative flavin-dependent L-lactate oxidase restricted to the intermembrane space 5739 744894 1.1.3.20 long-chain-alcohol oxidase mitochondrion - 5739 -, 724083 1.1.3.21 glycerol-3-phosphate oxidase mitochondrion - 5739 740092 1.1.3.37 D-arabinono-1,4-lactone oxidase mitochondrion - 5739 10663 1.1.3.40 D-mannitol oxidase mitochondrion - 5739 287629, 287631, 287632 1.1.3.40 D-mannitol oxidase mitochondrion not in mitochondria or peroxisomes 5739 287632 1.1.3.8 L-gulonolactone oxidase mitochondrion - 5739 389747 1.1.5.10 D-2-hydroxyacid dehydrogenase (quinone) mitochondrion - 5739 724205 1.1.5.13 (S)-2-hydroxyglutarate dehydrogenase mitochondrion - 5739 755031 1.1.5.3 glycerol-3-phosphate dehydrogenase mitochondrion - 5739 -, 287802, 287805, 287806, 287809, 287812, 287821, 287828, 287829, 685325, 687435, 689630, 740095, 740404, 740482, 740537, 741101, 760855, 762241, 762941, 763434 1.1.5.3 glycerol-3-phosphate dehydrogenase mitochondrion inner mitochondrial membrane. The mature protein has three transmembrane helices. The first membrane-spanning region coincides with the FAD site and thus this site is placed within the membrane. The calcium-binding region lies outside the membrane exposed to the cytosolic environment 5739 287831 1.1.5.3 glycerol-3-phosphate dehydrogenase mitochondrion located on the inner mitochondrial membrane 5739 695272 1.1.5.3 glycerol-3-phosphate dehydrogenase mitochondrion nuclear-encoded enzyme associated with the inner mitochondrial membrane 5739 690606 1.1.5.3 glycerol-3-phosphate dehydrogenase mitochondrion the enzyme is localized to the outer membrane of the mitochondria facing the cytoplasm 5739 713077 1.1.5.3 glycerol-3-phosphate dehydrogenase mitochondrion tightly bound to the outer surface of the inner mitochondrial membrane 5739 690607 1.1.5.4 malate dehydrogenase (quinone) mitochondrion - 5739 763119, 763412 1.1.5.4 malate dehydrogenase (quinone) mitochondrion not in cytosol, all analysed enzymes of the tricarboxylic acid –cycle are localised in the mitochondrion 5739 687185 1.1.99.1 choline dehydrogenase mitochondrion - 5739 389883, 389884, 389885, 389886, 688563 1.1.99.1 choline dehydrogenase mitochondrion inner membrane 5739 654440 1.1.99.1 choline dehydrogenase mitochondrion metabolic organization of betaine synthesis comparable to those of other vertebrates 5739 686202 1.1.99.14 glycolate dehydrogenase mitochondrion - 5739 656411, 688079 1.1.99.14 glycolate dehydrogenase mitochondrion by sucrose density gradient centrifugation 5739 -, 348289 1.1.99.2 L-2-hydroxyglutarate dehydrogenase mitochondrion - 5739 678483, 741137 1.1.99.2 L-2-hydroxyglutarate dehydrogenase mitochondrion bound to membrane 5739 657168 1.1.99.24 hydroxyacid-oxoacid transhydrogenase mitochondrion - 5739 -, 287797, 664967 1.1.99.39 D-2-hydroxyglutarate dehydrogenase mitochondrion - 5739 698932, 713271, 741050 1.1.99.6 D-lactate dehydrogenase (acceptor) mitochondrion - 5739 287839 1.10.3.11 ubiquinol oxidase (non-electrogenic) mitochondrion - 5739 -, 713936, 713939, 714297, 714299, 714309, 714998, 715002, 715410, 715441, 715963, 716585, 716591, 716720, 724413, 743451, 743543, 763851, 764782, 764800, 765051, 765531, 765548, 765550, 765552, 765558, 765807, 765865 1.11.1.11 L-ascorbate peroxidase mitochondrion - 5739 -, 697682, 698047, 765656 1.11.1.11 L-ascorbate peroxidase mitochondrion enzyme is closely associated with mitochondrial membranes 5739 743696 1.11.1.11 L-ascorbate peroxidase mitochondrion inside, either bound to the inner membrane or in the matrix 5739 439875 1.11.1.12 phospholipid-hydroperoxide glutathione peroxidase mitochondrion - 5739 394880, 394883, 658251, 672372, 673176, 673837, 674370, 674502, 674598, 676554, 686888, 696435, 697778, 742558 1.11.1.12 phospholipid-hydroperoxide glutathione peroxidase mitochondrion inner membrane 5739 394866 1.11.1.12 phospholipid-hydroperoxide glutathione peroxidase mitochondrion L-form of the enzyme 5739 673931 1.11.1.12 phospholipid-hydroperoxide glutathione peroxidase mitochondrion only in testicle 5739 673845 1.11.1.12 phospholipid-hydroperoxide glutathione peroxidase mitochondrion transgenic mice in which PHGPx is overexpressed solely in the mitochondrion. Mitochondria-specific GPx4 overexpression protects cardiac contractile function and preserves electron transport chain complex activities following ischemia/reperfusion 5739 686888 1.11.1.24 thioredoxin-dependent peroxiredoxin mitochondrion - 5739 -, 655661, 655981, 656491, 676075, 684680, 684853, 689642, 757915 1.11.1.24 thioredoxin-dependent peroxiredoxin mitochondrion after spermiation, PRDX2 localization becomes confined to the mitochondrial sheath of the sperm tail midpiece 5739 696569 1.11.1.24 thioredoxin-dependent peroxiredoxin mitochondrion localized to the mitochondrial matrix 5739 696346 1.11.1.24 thioredoxin-dependent peroxiredoxin mitochondrion MPX 5739 684996 1.11.1.24 thioredoxin-dependent peroxiredoxin mitochondrion Prx III 5739 655969 1.11.1.24 thioredoxin-dependent peroxiredoxin mitochondrion the nuclear-encoded protein TgTPx1/2 is trafficked to the apicoplast via a secretory route through the endoplasmic reticulum and to the mitochondrion via a non-secretory pathway comprising of translocon uptake. The signals for apicoplast and mitochondrial trafficking lie in the N-terminal 50 amino acids of the protein and are overlapping 5739 -, 757915 1.11.1.25 glutaredoxin-dependent peroxiredoxin mitochondrion - 5739 -, 752510, 754093 1.11.1.27 glutathione-dependent peroxiredoxin mitochondrion - 5739 700171 1.11.1.28 lipoyl-dependent peroxiredoxin mitochondrion the enzyme is preferentially located in mitochondrion 5739 -, 758317 1.11.1.3 fatty-acid peroxidase mitochondrion - 5739 439809 1.11.1.5 cytochrome-c peroxidase mitochondrion - 5739 -, 394614, 394617, 658807, 686388, 726385, 742669, 765121 1.11.1.5 cytochrome-c peroxidase mitochondrion Ccp1 5739 -, 725070 1.11.1.6 catalase mitochondrion - 5739 711009 1.11.1.6 catalase mitochondrion mainly in liver and kidney 5739 439797 1.11.1.8 iodide peroxidase mitochondrion - 5739 671397 1.11.1.8 iodide peroxidase mitochondrion Hashimoto‘s thyroiditis and oncocytic adenoma, are typically packed with mitochondria, display TPO reaction exclusivly in mitochondrial crisae 5739 658882 1.11.1.9 glutathione peroxidase mitochondrion - 5739 658792, 677190, 685439, 686868, 712067, 726239 1.11.1.9 glutathione peroxidase mitochondrion higher GPx activities are observed in cytosolic fractions than mitochondrial fractions in both the olfactory and liver tissues 5739 724102 1.13.11.18 persulfide dioxygenase mitochondrion - 5739 726227, 764794, 765046 1.13.11.40 arachidonate 8-lipoxygenase mitochondrion - 5739 439398 1.13.11.58 linoleate 9S-lipoxygenase mitochondrion - 5739 764893 1.13.11.63 beta-carotene 15,15'-dioxygenase mitochondrion - 5739 -, 745967 1.13.11.71 carotenoid-9',10'-cleaving dioxygenase mitochondrion - 5739 727353, 744161, 745345, 745753 1.13.99.1 inositol oxygenase mitochondrion - 5739 -, 745184, 745343, 745357 1.14.11.16 peptide-aspartate beta-dioxygenase mitochondrion - 5739 743376 1.14.11.2 procollagen-proline 4-dioxygenase mitochondrion 47.9% of total activity 5739 439220 1.14.11.65 [histone H3]-dimethyl-L-lysine9 demethylase mitochondrion enzyme LSD1 is present at the proximal region of the microsomal PGES-1 promoter 5739 744188 1.14.11.67 [histone H3]-trimethyl-L-lysine4 demethylase mitochondrion - 5739 700201 1.14.11.8 trimethyllysine dioxygenase mitochondrion - 5739 675029, 691166, 697027, 7019, 7020, 7021, 744681, 745961 1.14.11.8 trimethyllysine dioxygenase mitochondrion exclusively in 5739 652164 1.14.11.8 trimethyllysine dioxygenase mitochondrion mitochondrial matrix, product formation is limited by 6-N-trimethyllysine transport across the mitochondrial inner membrane 5739 686678 1.14.13.168 indole-3-pyruvate monooxygenase mitochondrion isoform YUC2 5739 765739 1.14.13.24 3-hydroxybenzoate 6-monooxygenase mitochondrion - 5739 -, 728255 1.14.13.32 albendazole monooxygenase mitochondrion - 5739 701177 1.14.13.39 nitric-oxide synthase (NADPH) mitochondrion - 5739 -, 440239, 671278, 699997, 701646 1.14.13.39 nitric-oxide synthase (NADPH) mitochondrion distinct enzyme not identical to other known isoforms 5739 658793 1.14.13.39 nitric-oxide synthase (NADPH) mitochondrion isozyme NOS1 contains a mitochondrial targeting signal 5739 673662 1.14.13.9 kynurenine 3-monooxygenase mitochondrion - 5739 -, 348514, 348515, 348516, 348517, 348518, 348519, 348520, 348522, 348523, 348524, 348528, 348530, 348531, 657495, 659985, 673130, 675745 1.14.13.9 kynurenine 3-monooxygenase mitochondrion promitochondrion 5739 348519 1.14.14.1 unspecific monooxygenase mitochondrion light mitochondrial fraction, in glucose-grown cells 5739 -, 672654 1.14.14.139 5beta-cholestane-3alpha,7alpha-diol 12alpha-hydroxylase mitochondrion - 5739 659575 1.14.14.16 steroid 21-monooxygenase mitochondrion - 5739 728828 1.14.14.18 heme oxygenase (biliverdin-producing) mitochondrion - 5739 -, 659620, 688881, 715599 1.14.14.18 heme oxygenase (biliverdin-producing) mitochondrion 77% activity in inner membrane, specifically associated with complex I, NADH: ubiquinone oxidoreductase 5739 438257 1.14.14.18 heme oxygenase (biliverdin-producing) mitochondrion liver, 7% of microsomal activity 5739 438250 1.14.14.24 vitamin D 25-hydroxylase mitochondrion - 5739 717750 1.14.14.24 vitamin D 25-hydroxylase mitochondrion heavy mitochondrial fraction 5739 717287 1.14.14.24 vitamin D 25-hydroxylase mitochondrion heavy mitochondrial fraction, highest activity found 5739 717287 1.14.14.25 cholesterol 24-hydroxylase mitochondrion of liver 5739 671173 1.14.14.32 17alpha-hydroxyprogesterone deacetylase mitochondrion - 5739 677795 1.14.14.91 trans-cinnamate 4-monooxygenase mitochondrion less than 5% of the activity 5739 348366 1.14.15.15 cholestanetriol 26-monooxygenase mitochondrion - 5739 439023, 439025, 439026, 439030, 439031, 439033, 439034, 439035, 439036, 671685, 671957, 674399, 674400, 712677, 713175, 725428, 735739, 735969, 736561 1.14.15.16 vitamin D3 24-hydroxylase mitochondrion - 5739 715412, 716973, 717838, 728214, 736086, 736545, 736557 1.14.15.18 calcidiol 1-monooxygenase mitochondrion - 5739 285537, 440101, 440104, 440105, 440106, 440107, 440109, 440110, 440111, 440112, 440113, 440114, 440115, 440116, 440121, 440122, 440123, 440124, 440128, 440129, 440131, 440132, 659943, 671685, 672857, 699086, 699219 1.14.15.4 steroid 11beta-monooxygenase mitochondrion - 5739 285386, 285387, 285389, 285390, 285391, 285392, 285393, 285397, 285399, 285402, 285403, 285404, 285405, 285407, 673923, 686928, 698085, 725828 1.14.15.4 steroid 11beta-monooxygenase mitochondrion CYP11 5739 285411 1.14.15.4 steroid 11beta-monooxygenase mitochondrion CYP11B1 in zona fasciculata, CYP11B2 in zona glomerulosa, new CYP11B3 5739 285410 1.14.15.4 steroid 11beta-monooxygenase mitochondrion CYP11B2 5739 712715 1.14.15.4 steroid 11beta-monooxygenase mitochondrion cytochrome P-45011beta in inner mitochondrial membrane, adrenodoxin reductase and adrenodoxin are loosely associated, inclusion of purified P-45011beta into phospholipid vesicles mimics the situation in vivo 5739 285394 1.14.15.4 steroid 11beta-monooxygenase mitochondrion from zona fasciculata 5739 285395, 285398, 285400, 285401 1.14.15.4 steroid 11beta-monooxygenase mitochondrion from zona glomerulosa 5739 285395, 285398, 285400, 285401 1.14.15.4 steroid 11beta-monooxygenase mitochondrion from zona glomerulosa and fasciculata 5739 285400 1.14.15.4 steroid 11beta-monooxygenase mitochondrion from zona reticularis 5739 285398, 285401 1.14.15.4 steroid 11beta-monooxygenase mitochondrion inner membrane, all 3 zones 5739 285396 1.14.15.4 steroid 11beta-monooxygenase mitochondrion reconstitution of cytochrome P45011beta, an intrinsic protein of inner membrane, into phospholipid vesicles via detergent dialysis procedure 5739 285398 1.14.15.4 steroid 11beta-monooxygenase mitochondrion the precursor form of the enzyme translocates into the matrix of the mitochondria, where it undergoes cleavage by mitochondrial processing peptidase to a mature form of approximately 54 kDa. Mature enzyme seems to translocate across the inner mitochondrial membrane a second time to finally reside in the intermembrane space 5739 726012 1.14.15.6 cholesterol monooxygenase (side-chain-cleaving) mitochondrion - 5739 659805, 687970, 688147, 703418, 703625, 704039, 705461, 725459, 744728 1.14.15.6 cholesterol monooxygenase (side-chain-cleaving) mitochondrion truncated form is nonmitochondrial 5739 705739 1.14.16.2 tyrosine 3-monooxygenase mitochondrion - 5739 746466 1.14.19.17 sphingolipid 4-desaturase mitochondrion - 5739 734604 1.14.19.17 sphingolipid 4-desaturase mitochondrion N-myristoylation targets dihydroceramide DELTA4-desaturase 1 to mitochondria 5739 733429 1.14.19.2 stearoyl-[acyl-carrier-protein] 9-desaturase mitochondrion a mitochondrial targeting peptide is predicted in TcSAD3 5739 744992 1.14.19.2 stearoyl-[acyl-carrier-protein] 9-desaturase mitochondrion predicted 5739 676555 1.14.19.31 acyl-lipid (7-3)-desaturase mitochondrion the localization of FADS2 in mitochondria, supports the involvement of this organelle in long-chain polyunsaturated fatty acid synthesis 5739 744864 1.14.19.44 acyl-CoA (8-3)-desaturase mitochondrion - 5739 734434 1.14.19.67 salutaridine synthase mitochondrion - 5739 393875, 393876 1.14.19.67 salutaridine synthase mitochondrion light mitochondrial fraction 5739 393876 1.14.99.22 ecdysone 20-monooxygenase mitochondrion - 5739 285457, 715821 1.14.99.22 ecdysone 20-monooxygenase mitochondrion membrane bound 5739 285450, 285453, 285456 1.14.99.22 ecdysone 20-monooxygenase mitochondrion mitochondrial isozyme 5739 285458 1.14.99.38 cholesterol 25-monooxygenase mitochondrion - 5739 727109 1.14.99.60 3-demethoxyubiquinol 3-hydroxylase mitochondrion - 5739 746696, 746956, 748142 1.14.99.60 3-demethoxyubiquinol 3-hydroxylase mitochondrion mitochondrial inner membrane protein 5739 748155 1.15.1.1 superoxide dismutase mitochondrion - 5739 -, 438153, 673161, 673321, 673792, 674446, 676295, 703644, 744281 1.15.1.1 superoxide dismutase mitochondrion Cu,Zn-SOD 5739 438162 1.15.1.1 superoxide dismutase mitochondrion in the light mitochondrial fraction 5739 745093 1.15.1.1 superoxide dismutase mitochondrion isozyme SODIII 5739 705498 1.15.1.1 superoxide dismutase mitochondrion mitMn-SOD 5739 688077 1.15.1.1 superoxide dismutase mitochondrion mitochondrial MnSOD isozyme mtMnSOD 5739 703780 1.15.1.1 superoxide dismutase mitochondrion Mn-SOD 5739 -, 438097, 438100, 438102, 438106, 438118, 438152, 438162 1.15.1.1 superoxide dismutase mitochondrion MnSOD 5739 713971 1.15.1.1 superoxide dismutase mitochondrion MnSOD-2 and MnSOD-3 5739 701484 1.15.1.1 superoxide dismutase mitochondrion SOD-I 5739 -, 438150 1.15.1.1 superoxide dismutase mitochondrion surrounding the central vacuole 5739 746055 1.15.1.1 superoxide dismutase mitochondrion the enzyme contains a highly conserved mitochondria-targeting signal 5739 714772 1.15.1.1 superoxide dismutase mitochondrion the enzyme contains an N-terminal mitochondrial targeting sequence 5739 706067 1.15.1.1 superoxide dismutase mitochondrion the enzyme possesses a unique 35 amino acid mitochondrial targeting sequence at the N-terminus 5739 705102 1.15.1.1 superoxide dismutase mitochondrion the enzyme sequence encodes 218 amino acids with a 20 amino acid mitochondrial targeting sequence in the N-terminus 5739 746564 1.15.1.1 superoxide dismutase mitochondrion two potential distinct mMnSOD isoforms presenting particular peptide signals 5739 703295 1.16.1.6 cyanocobalamin reductase mitochondrion - 5739 438037 1.16.1.7 ferric-chelate reductase (NADH) mitochondrion FRO7 5739 689790 1.16.98.B1 mitochondrial amidoxime reducing component mitochondrion - 5739 760877, 761442, 761666 1.17.1.9 formate dehydrogenase mitochondrion - 5739 741207 1.17.1.9 formate dehydrogenase mitochondrion of transgenic Arabidopsis and tobacco plants 5739 657097 1.17.3.2 xanthine oxidase mitochondrion - 5739 673811 1.17.4.1 ribonucleoside-diphosphate reductase mitochondrion enzyme may be specifically associated with mitochondria 5739 437975 1.17.4.1 ribonucleoside-diphosphate reductase mitochondrion incubation of respiring rat liver mitochondria with [14C]cytidine diphosphate leads to accumulation of radiolabeled deoxycytidine and thymidine nucleotides within the mitochondria 5739 727944 1.17.99.3 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase mitochondrion - 5739 659114 1.17.99.9 heme a synthase mitochondrion - 5739 -, 758753, 758779, 759472 1.18.1.2 ferredoxin-NADP+ reductase mitochondrion - 5739 285475, 285507, 285518, 285537 1.18.1.2 ferredoxin-NADP+ reductase mitochondrion mtFNR 5739 -, 716851 1.18.1.6 adrenodoxin-NADP+ reductase mitochondrion - 5739 285476, 285521, 285528, 285555, 659805, 718822, 719028, 719475, 719482, 719751, 719752, 719768, 719769, 719775, 720222, 727090, 744400, 745575, 746398 1.2.1.10 acetaldehyde dehydrogenase (acetylating) mitochondrion higher enzyme level in mitochondria from cells grown at pH 6.0 than in mitochondria from cells grown at pH 3.7 5739 739328 1.2.1.104 pyruvate dehydrogenase system mitochondrion - 5739 -, 348916, 348924, 348925, 348931, 348938, 348947, 348969, 348970, 348985, 655234, 656269, 656612, 676465, 687812, 695494, 700076, 710828, 712006, 758719, 758744, 758780, 758867, 759464, 759502, 759561, 759744, 759942, 759985, 759986, 760048, 760089, 763321, 763587, 94891, 94893 1.2.1.104 pyruvate dehydrogenase system mitochondrion located within the inner membrane matrix compartment 5739 94881 1.2.1.104 pyruvate dehydrogenase system mitochondrion subunit E1, EC 1.2.4.1, of whole enzyme complex has lower affinity to membrane than E2, EC 1.8.1.4 5739 348927 1.2.1.105 2-oxoglutarate dehydrogenase system mitochondrion - 5739 348910, 349002, 349005, 349006, 349008, 349021, 349033, 349034, 349035, 349036, 349037, 349038, 349040, 349042, 654854, 655234, 656174, 656265, 672202, 672280, 673654, 676088, 676319, 685238, 685378, 689950, 700075, 700476, 700812, 758628, 758745, 758854, 758867, 759216, 759561, 759827, 759969, 763003 1.2.1.105 2-oxoglutarate dehydrogenase system mitochondrion alpha-ketoglutarate dehydrogenase is heterogeneously distributed in mitochondria within individual astrocytes originating either from cerebellum or cerebral cortex 5739 673870 1.2.1.12 glyceraldehyde-3-phosphate dehydrogenase (phosphorylating) mitochondrion - 5739 -, 287913, 742889 1.2.1.12 glyceraldehyde-3-phosphate dehydrogenase (phosphorylating) mitochondrion enzyme form E6.8, E8.5 and E9.0 5739 287913 1.2.1.16 succinate-semialdehyde dehydrogenase [NAD(P)+] mitochondrion - 5739 -, 288048 1.2.1.16 succinate-semialdehyde dehydrogenase [NAD(P)+] mitochondrion of non-synaptic origin 5739 -, 288050 1.2.1.18 malonate-semialdehyde dehydrogenase (acetylating) mitochondrion - 5739 288055, 689379, 690142 1.2.1.19 aminobutyraldehyde dehydrogenase mitochondrion - 5739 288080 1.2.1.22 lactaldehyde dehydrogenase mitochondrion - 5739 697876 1.2.1.24 succinate-semialdehyde dehydrogenase (NAD+) mitochondrion - 5739 288152, 288161, 654852, 656587, 657160, 689379, 694354, 700887, 741570, 763472 1.2.1.24 succinate-semialdehyde dehydrogenase (NAD+) mitochondrion matrix 5739 288156, 288173 1.2.1.25 branched-chain alpha-keto acid dehydrogenase system mitochondrion - 5739 -, 762713, 762780, 763378, 763513 1.2.1.27 methylmalonate-semialdehyde dehydrogenase (CoA-acylating) mitochondrion - 5739 698095 1.2.1.27 methylmalonate-semialdehyde dehydrogenase (CoA-acylating) mitochondrion mitochondrial targeting sequence 5739 390221, 390223 1.2.1.28 benzaldehyde dehydrogenase (NAD+) mitochondrion - 5739 700759 1.2.1.3 aldehyde dehydrogenase (NAD+) mitochondrion - 5739 348695, 348699, 348700, 348712, 348713, 348714, 348715, 348718, 654424, 655948, 656837, 656901, 673505, 675118, 676317, 684721, 689379, 690142, 690896, 691775, 692623, 692780, 693339, 693766, 695139, 697280, 697845, 698765, 711583, 711625, 711836, 711924, 712375, 712435, 712497, 724459, 724716, 740442, 741147, 762790 1.2.1.3 aldehyde dehydrogenase (NAD+) mitochondrion ALDH II 5739 691867 1.2.1.3 aldehyde dehydrogenase (NAD+) mitochondrion enzyme ALDH-2 5739 348686, 348694 1.2.1.3 aldehyde dehydrogenase (NAD+) mitochondrion enzyme I is localized in the matrix 5739 348682 1.2.1.3 aldehyde dehydrogenase (NAD+) mitochondrion expression of ALDH2 increased during development 5739 677208 1.2.1.3 aldehyde dehydrogenase (NAD+) mitochondrion highest activity in mitochondria 5739 711651 1.2.1.3 aldehyde dehydrogenase (NAD+) mitochondrion highest expression of ALDH2 at the beginning and the end of development 5739 677208 1.2.1.3 aldehyde dehydrogenase (NAD+) mitochondrion isoenzyme ALDH2 5739 348710 1.2.1.3 aldehyde dehydrogenase (NAD+) mitochondrion isoenzyme I and II 5739 348705, 348791 1.2.1.3 aldehyde dehydrogenase (NAD+) mitochondrion isoform ALDH2 5739 712069 1.2.1.31 L-aminoadipate-semialdehyde dehydrogenase mitochondrion - 5739 690142 1.2.1.36 retinal dehydrogenase mitochondrion - 5739 390268, 690142, 762790 1.2.1.38 N-acetyl-gamma-glutamyl-phosphate reductase mitochondrion - 5739 390281, 390284, 390289 1.2.1.38 N-acetyl-gamma-glutamyl-phosphate reductase mitochondrion soluble matrix enzyme 5739 1730 1.2.1.41 glutamate-5-semialdehyde dehydrogenase mitochondrion - 5739 390302, 390303, 689379, 690142 1.2.1.47 4-trimethylammoniobutyraldehyde dehydrogenase mitochondrion - 5739 690142 1.2.1.48 long-chain-aldehyde dehydrogenase mitochondrion - 5739 347796, 390318, 390323 1.2.1.5 aldehyde dehydrogenase [NAD(P)+] mitochondrion - 5739 -, 348786, 348788, 655423, 684721, 689379, 690142, 698095, 724720, 740279, 762546 1.2.1.5 aldehyde dehydrogenase [NAD(P)+] mitochondrion enzyme form ALDH5 5739 348783 1.2.1.5 aldehyde dehydrogenase [NAD(P)+] mitochondrion enzyme I and II 5739 348789 1.2.1.5 aldehyde dehydrogenase [NAD(P)+] mitochondrion membrane 5739 348781 1.2.1.51 pyruvate dehydrogenase (NADP+) mitochondrion - 5739 288278, 288282, 288283, 288284, 654355 1.2.1.8 betaine-aldehyde dehydrogenase mitochondrion - 5739 390344, 390362, 390363, 689573, 698095, 740512, 762849 1.2.1.8 betaine-aldehyde dehydrogenase mitochondrion 5% of the activity in mitochondrial matrix, 95% of the activity in cytosol 5739 390347 1.2.1.8 betaine-aldehyde dehydrogenase mitochondrion matrix. Cytoplasmic and mitochondrial enzymes are products of the same gene 5739 390357 1.2.1.8 betaine-aldehyde dehydrogenase mitochondrion the enzyme is approximately equally distributed between the inner membrane and the intermembrane plus matrix fraction 5739 390343 1.2.1.88 L-glutamate gamma-semialdehyde dehydrogenase mitochondrion - 5739 -, 392042, 698095, 698936, 740744, 763363 1.2.1.88 L-glutamate gamma-semialdehyde dehydrogenase mitochondrion exclusively 5739 654887 1.2.1.88 L-glutamate gamma-semialdehyde dehydrogenase mitochondrion matrix 5739 392039, 392048, 392049, 392052, 392054, 392064 1.2.3.1 aldehyde oxidase mitochondrion light and heavy mitochondrial fractions 5739 390395 1.2.4.1 pyruvate dehydrogenase (acetyl-transferring) mitochondrion - 5739 -, 348935, 348951, 348955, 348968, 348974, 654462, 657000, 657068, 684365, 697756, 700236, 712851, 740976, 741339, 741340, 758719, 759464, 760048, 763321, 763587 1.2.4.2 oxoglutarate dehydrogenase (succinyl-transferring) mitochondrion - 5739 349000, 349009, 349010, 656572, 656595, 656596, 673146, 689172, 696124, 697874, 699606, 699660, 740480, 740535, 759216, 759969, 763067 1.2.4.2 oxoglutarate dehydrogenase (succinyl-transferring) mitochondrion enzyme E1o contains mitochondrial targeting sequence 5739 656835 1.2.4.2 oxoglutarate dehydrogenase (succinyl-transferring) mitochondrion subsarcolemmal 5739 654699 1.2.4.4 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) mitochondrion - 5739 349057, 349059, 349060, 349061, 349062, 349063, 349064, 349070, 349074, 349075, 349079, 654466, 655560, 656045, 657305, 657370, 673938, 675567, 687542, 687543, 712648, 712934, 713078, 763049 1.2.4.4 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) mitochondrion inner membrane matrix compartment 5739 94884 1.2.7.4 anaerobic carbon-monoxide dehydrogenase mitochondrion - 5739 685228 1.21.1.1 iodotyrosine deiodinase mitochondrion - 5739 704368 1.21.99.3 thyroxine 5-deiodinase mitochondrion isozyme type III 5739 644798 1.21.99.4 thyroxine 5'-deiodinase mitochondrion - 5739 644806 1.3.1.1 dihydropyrimidine dehydrogenase (NAD+) mitochondrion low content 5739 349141 1.3.1.10 enoyl-[acyl-carrier-protein] reductase (NADPH, Si-specific) mitochondrion - 5739 656515 1.3.1.104 enoyl-[acyl-carrier-protein] reductase (NADPH) mitochondrion - 5739 763551 1.3.1.124 2,4-dienoyl-CoA reductase [(3E)-enoyl-CoA-producing] mitochondrion - 5739 -, 390707, 390709, 390712, 390714, 390715, 390716, 390718, 390719, 390722, 654376, 656286, 685405, 690046, 700872, 758746, 759137, 759825, 760087, 760176 1.3.1.22 3-oxo-5alpha-steroid 4-dehydrogenase (NADP+) mitochondrion - 5739 396212 1.3.1.24 biliverdin reductase mitochondrion - 5739 712793 1.3.1.34 2,4-dienoyl-CoA reductase [(2E)-enoyl-CoA-producing] mitochondrion - 5739 390710, 652365, 657265, 759709 1.3.1.38 trans-2-enoyl-CoA reductase (NADPH) mitochondrion - 5739 390758, 706445, 740376 1.3.1.44 trans-2-enoyl-CoA reductase (NAD+) mitochondrion - 5739 390806, 390810, 656287 1.3.1.6 fumarate reductase (NADH) mitochondrion - 5739 -, 390843, 657430, 684492, 689320, 695392, 710873, 713389, 725318 1.3.1.6 fumarate reductase (NADH) mitochondrion isozymes FRDm1 and FRDm2 5739 656280 1.3.1.7 meso-tartrate dehydrogenase mitochondrion - 5739 349356 1.3.1.8 acyl-CoA dehydrogenase (NADP+) mitochondrion - 5739 390758, 390855, 390856, 390857, 390870, 685980 1.3.1.8 acyl-CoA dehydrogenase (NADP+) mitochondrion soluble fraction of mitochondria 5739 390810 1.3.2.3 L-galactonolactone dehydrogenase mitochondrion - 5739 -, 390902, 390903, 390905, 676489, 676685, 676754, 686713, 689590, 698838, 700833, 713264, 725468, 726197, 746068 1.3.2.3 L-galactonolactone dehydrogenase mitochondrion associated membrane protein 5739 390907 1.3.2.3 L-galactonolactone dehydrogenase mitochondrion GalLDH from pepper fruits is attached to the mitochondrial membrane through, at least, a very short transmembrane domain of 20 amino acids, with the rest of the protein structure being peripheral 5739 746414 1.3.2.3 L-galactonolactone dehydrogenase mitochondrion inner membrane 5739 656407 1.3.2.3 L-galactonolactone dehydrogenase mitochondrion intrinsic in inner membrane 5739 390904 1.3.2.3 L-galactonolactone dehydrogenase mitochondrion L-GalLDH is attached to complex I of the mitochondrial electron transport chain 5739 745635 1.3.2.3 L-galactonolactone dehydrogenase mitochondrion soluble 5739 390906 1.3.2.3 L-galactonolactone dehydrogenase mitochondrion the enzyme contains a mitochondrial targeting sequence 5739 746064 1.3.2.3 L-galactonolactone dehydrogenase mitochondrion the enzyme contains a putative mitochondrial targeting signal peptide 5739 746491 1.3.3.12 L-galactonolactone oxidase mitochondrion - 5739 389744, 389747 1.3.3.3 coproporphyrinogen oxidase mitochondrion - 5739 -, 390949, 390952, 390966, 390967, 390971, 390974, 390978, 390980, 656432, 672533, 677031, 684418, 697204, 712835, 724548, 744139, 746121, 763670, 763898, 764113, 764514, 765391, 765393 1.3.3.3 coproporphyrinogen oxidase mitochondrion CPX2 5739 676504 1.3.3.3 coproporphyrinogen oxidase mitochondrion highest recovery of enzyme activity by digitonin treatment of mitochondria 5739 390969 1.3.3.3 coproporphyrinogen oxidase mitochondrion human enzyme is imported in isolated rat mitochondria, heme inhibits the mitochondrial import of the enzyme completely at 0.02 mM concentration 5739 655119 1.3.3.3 coproporphyrinogen oxidase mitochondrion in digitonin-treated mitochondria from animals exposed to hexachlorobenzene, coproporphyrinogen oxidase is free in the mitochondrial intermembrane space, whereas in normal mitochondria, 30%-50% remain anchored to the inner membrane 5739 698371 1.3.3.3 coproporphyrinogen oxidase mitochondrion located within intermembrane space 5739 390943, 390949, 390968, 390969 1.3.3.4 protoporphyrinogen oxidase mitochondrion - 5739 390981, 390982, 390990, 390991, 390992, 390993, 390997, 390999, 391003, 391006, 391009, 671294, 672962, 676307, 690193, 711307, 724557, 726346, 744734, 745140, 745787, 746243, 764954, 764962, 765423 1.3.3.4 protoporphyrinogen oxidase mitochondrion greatly increased Protox activity in transgenic rice as compared to wild-type 5739 676642 1.3.3.4 protoporphyrinogen oxidase mitochondrion isozyme PPO2, outer surface of the inner membrane 5739 655341 1.3.3.4 protoporphyrinogen oxidase mitochondrion lipid bilayer of inner membrane 5739 390985, 390994, 390995, 391001 1.3.3.4 protoporphyrinogen oxidase mitochondrion membrane bound 5739 390986, 390998, 390999, 391008 1.3.3.4 protoporphyrinogen oxidase mitochondrion mitochondrial isozyme 5739 655018 1.3.3.4 protoporphyrinogen oxidase mitochondrion no enzyme found 5739 391014 1.3.3.4 protoporphyrinogen oxidase mitochondrion PPO is localized in human mitochondria, but is not found in mitochondria when expressed in rice leaves 5739 689568 1.3.3.4 protoporphyrinogen oxidase mitochondrion PPO2 5739 671892 1.3.3.4 protoporphyrinogen oxidase mitochondrion prominent increase of Protox activity in transgenic rice plant lines TTS3 and TTS4 relative to the wild-type, increase is lower than that in transgenic line M4 5739 676308 1.3.3.5 bilirubin oxidase mitochondrion - 5739 391020 1.3.3.5 bilirubin oxidase mitochondrion inner and outer membrane 5739 391023 1.3.5.1 succinate dehydrogenase mitochondrion - 5739 -, 2877, 391080, 391081, 391087, 391138, 391146, 391156, 391165, 391179, 391180, 391189, 391191, 654092, 654172, 654199, 654444, 654819, 656178, 656275, 656276, 656605, 657158, 671041, 672346, 672379, 673796, 673871, 685306, 686054, 686399, 686400, 686687, 686749, 687015, 687110, 687965, 687966, 688533, 688882, 689109, 689159, 689285, 689710, 695601, 697000, 698668, 699579, 711232, 712582, 712595, 725316, 726243, 762781, 762805, 762895 1.3.5.1 succinate dehydrogenase mitochondrion based on the presence of a predicted mitochondrial targeting peptides the protein is imported into mitochondria 5739 697000 1.3.5.1 succinate dehydrogenase mitochondrion cytoplasmic side of inner membrane 5739 391153 1.3.5.1 succinate dehydrogenase mitochondrion enzyme isolated from mitochondria 5739 698658 1.3.5.1 succinate dehydrogenase mitochondrion inner membrane 5739 391081, 391114, 391135 1.3.5.1 succinate dehydrogenase mitochondrion matrix side 5739 391115 1.3.5.1 succinate dehydrogenase mitochondrion mitochondria from L3 larvae and adult Ascaris suum muscle are prepared 5739 689320 1.3.5.1 succinate dehydrogenase mitochondrion mitochondria from Septoria nodorum are prepared 5739 696701 1.3.5.1 succinate dehydrogenase mitochondrion mitochondrion from bovine heart are prepared 5739 696701 1.3.5.1 succinate dehydrogenase mitochondrion of the adult parasitic nematode and of free living second stage larvae 5739 391128, 391138 1.3.5.1 succinate dehydrogenase mitochondrion submitochondrial particles are prepared from frozen muscles of Ascaris suum adult female worms 5739 700074 1.3.5.1 succinate dehydrogenase mitochondrion the effect of papyriferic acid on the oxidation of succinate by SDH is examined in mitochondrial preparations from livers 5739 699137 1.3.5.2 dihydroorotate dehydrogenase (quinone) mitochondrion - 5739 390921, 390923, 390926, 390929, 391224, 391226, 391229, 391233, 391235, 711913, 712682, 713150, 725919, 725920, 740478, 741097, 762769, 763072 1.3.5.2 dihydroorotate dehydrogenase (quinone) mitochondrion a class 2 mitochondrial enzyme 5739 712147 1.3.5.2 dihydroorotate dehydrogenase (quinone) mitochondrion cardiolipin enhances the interaction of truncated DHODH with lipid bilayers, but the presence of the transmembrane domain in human DHODH is necessary for stable binding to and securing its location at the outer surface of the inner mitochondrial membrane 5739 763527 1.3.5.2 dihydroorotate dehydrogenase (quinone) mitochondrion inner membrane 5739 390922, 390924, 390925, 390929, 391221 1.3.5.2 dihydroorotate dehydrogenase (quinone) mitochondrion integral protein 5739 390924 1.3.5.2 dihydroorotate dehydrogenase (quinone) mitochondrion membrane 5739 391223 1.3.5.2 dihydroorotate dehydrogenase (quinone) mitochondrion outer membrane of 5739 656088 1.3.7.12 red chlorophyll catabolite reductase mitochondrion - 5739 676913, 700695 1.3.7.12 red chlorophyll catabolite reductase mitochondrion in young seedlings and in response to stress, RCCR is somewhat localized to mitochondria 5739 699585 1.3.7.12 red chlorophyll catabolite reductase mitochondrion soluble protein of chloroplasts, in young Arabidopsis seedlings also associated with mitochondria 5739 671354 1.3.8.1 short-chain acyl-CoA dehydrogenase mitochondrion - 5739 391203, 391256, 391272, 391283, 391285, 391287, 391290, 656811, 686920, 687026, 689006, 689021, 700228, 712126, 712650, 713043, 725641 1.3.8.1 short-chain acyl-CoA dehydrogenase mitochondrion subunits of the SCAD enzyme are synthesised in the cytosol as precursor proteins that are then imported into the mitochondrial matrix 5739 712123 1.3.8.4 isovaleryl-CoA dehydrogenase mitochondrion - 5739 -, 391199, 391200, 391201, 391202, 391203, 391204, 391205, 391206, 391207, 391208, 391212, 391213, 391214, 391216, 391219, 685654, 713271, 724512, 724826, 725099, 742009 1.3.8.5 short-chain 2-methylacyl-CoA dehydrogenase mitochondrion - 5739 12302, 12304, 391203, 391252, 391254 1.3.8.6 glutaryl-CoA dehydrogenase (ETF) mitochondrion - 5739 391406, 391408, 391410, 391412, 391414, 654767, 656440, 688177, 689338, 713046, 762752 1.3.8.6 glutaryl-CoA dehydrogenase (ETF) mitochondrion mitochondrial matrix 5739 742629 1.3.8.6 glutaryl-CoA dehydrogenase (ETF) mitochondrion mitochondrial proteins interacting directly with GCDH are, among others, dihydrolipoamide S-succinyltransferase involved in the formation of glutaryl-CoA, and the beta-subunit of the electron transfer flavoprotein serving as electron acceptor 5739 743652 1.3.8.7 medium-chain acyl-CoA dehydrogenase mitochondrion - 5739 391282, 391338, 655491, 657278, 672585, 685352, 685980, 687009, 688185, 689196, 690129, 697048, 700546 1.3.8.8 long-chain acyl-CoA dehydrogenase mitochondrion - 5739 391047, 391199, 391203, 391265, 391268, 391270, 391271, 391272, 391275, 391282, 391298, 391325, 391361, 394642, 394650, 394651, 394652, 394653, 394659, 394665, 656692, 671584, 684281, 684346, 688181, 689761, 699301, 700233, 700578, 710755, 713044 1.3.8.8 long-chain acyl-CoA dehydrogenase mitochondrion inner mitochondrial membrane 5739 689007 1.3.8.8 long-chain acyl-CoA dehydrogenase mitochondrion MTE-II 5739 671275 1.3.8.9 very-long-chain acyl-CoA dehydrogenase mitochondrion - 5739 689339, 710755, 723908, 741280, 741392, 763263 1.3.98.1 dihydroorotate dehydrogenase (fumarate) mitochondrion - 5739 675478 1.3.98.1 dihydroorotate dehydrogenase (fumarate) mitochondrion inner membrane 5739 391222 1.3.98.3 coproporphyrinogen dehydrogenase mitochondrion recombinant AtHEMN1-GFP fusion protein is targeted to mitochondria 5739 746121 1.4.1.2 glutamate dehydrogenase mitochondrion - 5739 -, 391546, 391558, 391561, 391572, 676690, 685127, 688076, 688640, 689705, 698996, 700651, 726119, 726164, 726245, 760442, 763438 1.4.1.2 glutamate dehydrogenase mitochondrion four GDH deduced amino acid sequences possess the predicted mitochondrial target peptides, 18 amino acid residues at the N-terminus 5739 -, 725797 1.4.1.2 glutamate dehydrogenase mitochondrion GLUD2 is specifically targeted to mitochondria, whereas GLUD1 is localized in mitochondria and cytoplasm 5739 689705 1.4.1.2 glutamate dehydrogenase mitochondrion in mature flag leaves, GDH is localized in the mitochondria of the phloem companion cells, increases in senescensing flag leaves 5739 663075 1.4.1.2 glutamate dehydrogenase mitochondrion preferentially localized in the mitochondria of phloem companion cells in both leaves and flowers 5739 657131 1.4.1.2 glutamate dehydrogenase mitochondrion the N-terminal peptide preceding domain I is a mitochondrial targeting signal, the predicted cleavage site is Leu17-Leu18 followed by an potassium coordination site (Ser27, Ile30) 5739 763098 1.4.1.2 glutamate dehydrogenase mitochondrion the N-terminal peptide preceding domain I is a mitochondrial targeting signal, with a predicted cleavage site for mitochondrial processing peptidase (MPP) at Leu17-Leu18 that is followed by an unexpected potassium coordination site (Ser27, Ile30) 5739 763098 1.4.1.27 glycine cleavage system mitochondrion - 5739 743023, 743266, 758693, 758695, 758983, 759439, 759446, 759767, 759926, 759959 1.4.1.27 glycine cleavage system mitochondrion associated to inner membrane 5739 759438 1.4.1.27 glycine cleavage system mitochondrion mitochondrion-like organelle 5739 760050 1.4.1.3 glutamate dehydrogenase [NAD(P)+] mitochondrion - 5739 -, 391541, 391600, 391604, 391605, 391631, 656251, 673616, 700428, 712827, 725866, 725956, 742455, 743352, 743454, 762585, 762783, 762936, 763376, 763674 1.4.1.3 glutamate dehydrogenase [NAD(P)+] mitochondrion GDH2 5739 712431 1.4.1.3 glutamate dehydrogenase [NAD(P)+] mitochondrion more than two third of GLDH serum originates from mitochondria 5739 684262 1.4.1.4 glutamate dehydrogenase (NADP+) mitochondrion - 5739 -, 763355 1.4.3.1 D-aspartate oxidase mitochondrion - 5739 391726 1.4.3.2 L-amino-acid oxidase mitochondrion - 5739 391789, 391802 1.4.3.21 primary-amine oxidase mitochondrion - 5739 710834 1.4.3.22 diamine oxidase mitochondrion mainly in cytoplasm but to a considerable extent also in mitochondrial fraction 5739 391922 1.4.3.3 D-amino-acid oxidase mitochondrion matrix 5739 391840 1.4.3.4 monoamine oxidase mitochondrion - 5739 394548, 394549, 394554, 394557, 394558, 394563, 394565, 394566, 394568, 394570, 394574, 394575, 394576, 394580, 394581, 394583, 394585, 394587, 655523, 655781, 656607, 656857, 657397, 672531, 672541, 672601, 673203, 675099, 675287, 675304, 684241, 684263, 684770, 685517, 685535, 685556, 686527, 688063, 688257, 688268, 689177, 701958, 702669, 702900, 703627, 711407, 711424, 711437, 711442, 711446, 711447, 711449, 711912, 712076, 712683, 712684, 713198, 713457, 724524, 725620, 742357, 742989, 743126, 743640 1.4.3.4 monoamine oxidase mitochondrion external membrane 5739 764270 1.4.3.4 monoamine oxidase mitochondrion intra- and intermitochondrial membrane 5739 394573 1.4.3.4 monoamine oxidase mitochondrion outer membrane 5739 655288, 655583, 656469, 765139 1.4.3.4 monoamine oxidase mitochondrion outer membrane, the enzyme has a transmembrane helix at the C-terminal region 5739 656536 1.4.3.4 monoamine oxidase mitochondrion outer mitochondrial membrane 5739 725675 1.4.4.2 glycine dehydrogenase (aminomethyl-transferring) mitochondrion - 5739 391978, 391979, 391982, 391984, 391988, 391990, 391993, 391996, 391997, 392000, 688072, 688637, 689575, 697916, 713311, 725016, 763473, 765452 1.4.7.1 glutamate synthase (ferredoxin) mitochondrion - 5739 700740 1.5.1.15 methylenetetrahydrofolate dehydrogenase (NAD+) mitochondrion - 5739 392080, 392085, 392086, 392087, 392088, 392090, 392091, 392092, 654885, 656129, 671588, 756334, 756337, 757773, 765840 1.5.1.2 pyrroline-5-carboxylate reductase mitochondrion - 5739 392114, 392138, 740763, 762610, 762678, 762877 1.5.1.2 pyrroline-5-carboxylate reductase mitochondrion matrix space 5739 392120 1.5.1.3 dihydrofolate reductase mitochondrion - 5739 742518, 765575 1.5.1.3 dihydrofolate reductase mitochondrion the source of mitochondrial DHFR activity is parental DHFR in mouse 5739 742518 1.5.1.3 dihydrofolate reductase mitochondrion the source of mitochondrial DHFR activity is parental DHFR in rat 5739 742518 1.5.1.5 methylenetetrahydrofolate dehydrogenase (NADP+) mitochondrion - 5739 -, 392328, 701402, 742856, 756334, 757773 1.5.1.5 methylenetetrahydrofolate dehydrogenase (NADP+) mitochondrion MIS1 5739 701402 1.5.1.5 methylenetetrahydrofolate dehydrogenase (NADP+) mitochondrion the enzyme behaves as a tightly associated peripheral membrane protein of the mitochondrial inner membrane 5739 725456 1.5.1.6 formyltetrahydrofolate dehydrogenase mitochondrion - 5739 712450, 724712 1.5.1.6 formyltetrahydrofolate dehydrogenase mitochondrion comparable amounts of cytosolic and mitochondrial FDH in retinal tissues 5739 392355 1.5.1.6 formyltetrahydrofolate dehydrogenase mitochondrion small amount, 0.4% of total liver dehydrogenase activity 5739 392336 1.5.1.8 saccharopine dehydrogenase (NADP+, L-lysine-forming) mitochondrion - 5739 -, 392359, 392363, 392364, 392365 1.5.1.9 saccharopine dehydrogenase (NAD+, L-glutamate-forming) mitochondrion - 5739 -, 392363, 392364, 392365 1.5.1.9 saccharopine dehydrogenase (NAD+, L-glutamate-forming) mitochondrion mitochondrial matrix 5739 392366 1.5.3.10 dimethylglycine oxidase mitochondrion - 5739 655336 1.5.3.4 N6-methyl-lysine oxidase mitochondrion 60% of the activity in rat kidney is present in the mitochondrial fraction 5739 392428 1.5.3.4 N6-methyl-lysine oxidase mitochondrion exclusive localization 5739 392427 1.5.3.7 L-pipecolate oxidase mitochondrion - 5739 742960 1.5.3.7 L-pipecolate oxidase mitochondrion matrix protein 5739 392473 1.5.3.7 L-pipecolate oxidase mitochondrion soluble fraction 5739 392477 1.5.5.1 electron-transferring-flavoprotein dehydrogenase mitochondrion - 5739 -, 392479, 392481, 392483, 392486, 392487, 392488, 392492, 654538, 676412, 713271, 742306, 743249 1.5.5.1 electron-transferring-flavoprotein dehydrogenase mitochondrion ETFDH is an inner mitochondrial membrane localized enzyme 5739 705748 1.5.5.1 electron-transferring-flavoprotein dehydrogenase mitochondrion inner mitochondrial membrane 5739 676828, 677116 1.5.5.1 electron-transferring-flavoprotein dehydrogenase mitochondrion Magnaporthe oryzae ETF and ETFDH co-localization with the mitochondrial marker ATP1-RFP, but almost not with the peroxysomal marker PTS1-RFP 5739 743809 1.5.5.2 proline dehydrogenase mitochondrion transcriptional induction of the enzyme causes changes in expression levels of other mitochondrial enzymes. Activity of glutamate dehydrogenase is substantially increased, and activity of D-lactate dehydrogenase is increased 5739 742487 1.5.5.3 hydroxyproline dehydrogenase mitochondrion - 5739 741620, 741999, 742832 1.5.8.3 sarcosine dehydrogenase mitochondrion - 5739 392494, 392496, 392497, 392498, 392499, 392500, 392501, 392506, 392507, 392508 1.5.8.4 dimethylglycine dehydrogenase mitochondrion - 5739 392496, 392498, 392499, 392500, 392501, 392507, 392508, 392516, 392520, 392524, 392525, 392526, 392528, 657277, 687135, 741817, 742499, 743378 1.5.99.12 cytokinin dehydrogenase mitochondrion - 5739 655490 1.5.99.12 cytokinin dehydrogenase mitochondrion enzyme OsCKX2 5739 656629 1.5.99.12 cytokinin dehydrogenase mitochondrion enzymes AtCKX1 and AtCKX3 5739 656629 1.5.99.B2 proline dehydrogenase (acceptor) mitochondrion - 5739 392559, 392562, 392563, 392564, 392566, 392568, 675953, 684911, 685248, 686651, 687234, 687550, 689071, 711615 1.5.99.B2 proline dehydrogenase (acceptor) mitochondrion matrix side of inner membrane 5739 392558 1.5.99.B2 proline dehydrogenase (acceptor) mitochondrion outer phase of inner membrane 5739 392561 1.6.1.1 NAD(P)+ transhydrogenase (Si-specific) mitochondrion inside-out submitochondrial particles 5739 392593 1.6.1.2 NAD(P)+ transhydrogenase (Re/Si-specific) mitochondrion - 5739 -, 686316, 724953, 741801, 742241, 742520, 742631, 742757, 742841, 742857, 742893, 743045 1.6.1.2 NAD(P)+ transhydrogenase (Re/Si-specific) mitochondrion inner mitochondrial membrane 5739 711287 1.6.1.3 NAD(P)+ transhydrogenase mitochondrion - 5739 724104 1.6.2.2 cytochrome-b5 reductase mitochondrion - 5739 -, 394252, 724801, 742561, 743591 1.6.2.2 cytochrome-b5 reductase mitochondrion N-myristoylation is required for correct targeting 5739 674998 1.6.2.2 cytochrome-b5 reductase mitochondrion outer membrane enzyme complex 5739 394241 1.6.2.4 NADPH-hemoprotein reductase mitochondrion - 5739 285452, 288596, 288597, 288598, 743656 1.6.2.6 leghemoglobin reductase mitochondrion - 5739 394002 1.6.3.1 NAD(P)H oxidase (H2O2-forming) mitochondrion - 5739 696745 1.6.3.1 NAD(P)H oxidase (H2O2-forming) mitochondrion NOX4 5739 724778 1.6.5.10 NADPH dehydrogenase (quinone) mitochondrion CBR4 is a mitochondrial matrix protein, its N-terminal signal is not cleaved 5739 696015 1.6.5.2 NAD(P)H dehydrogenase (quinone) mitochondrion - 5739 394350, 394366, 715428, 724356 1.6.5.4 monodehydroascorbate reductase (NADH) mitochondrion - 5739 392741, 392747, 656980, 676536, 743842 1.6.5.4 monodehydroascorbate reductase (NADH) mitochondrion outer mitochondrial membrane 5739 392734, 392745 1.6.5.5 NADPH:quinone reductase mitochondrion - 5739 765788 1.6.5.6 p-benzoquinone reductase (NADPH) mitochondrion highest specific activity 5739 288623 1.6.5.9 NADH:quinone reductase (non-electrogenic) mitochondrion - 5739 392700, 714884, 723905, 764248 1.6.5.9 NADH:quinone reductase (non-electrogenic) mitochondrion after import, isoform NDA is intramitochondrially sorted to the matrix side of the inner membrane, whereas isoform NDB becomes exposed to the intermembrane space 5739 -, 716532 1.6.5.9 NADH:quinone reductase (non-electrogenic) mitochondrion alternative NADH dehydrogenase activity is located exclusively at the external face of the mitochondrial inner membrane 5739 677272 1.6.5.9 NADH:quinone reductase (non-electrogenic) mitochondrion internal surface of the inner mitochondrial membrane 5739 743895 1.6.5.9 NADH:quinone reductase (non-electrogenic) mitochondrion Ndi1 protein from Saccharomyces cerevisiae is a monotopic membrane protein, directed to the matrix 5739 726384 1.7.1.10 hydroxylamine reductase (NADH) mitochondrion - 5739 394436, 394437 1.7.1.10 hydroxylamine reductase (NADH) mitochondrion firmly attached to membrane 5739 394436, 394438 1.7.1.14 nitric oxide reductase [NAD(P)+, nitrous oxide-forming] mitochondrion - 5739 -, 688826, 713937 1.7.1.14 nitric oxide reductase [NAD(P)+, nitrous oxide-forming] mitochondrion isoform P450nor1 5739 714517 1.7.1.6 azobenzene reductase mitochondrion activity higher in crude outer mitochondrial membrane fraction than in mitoplast and intact mitochondria 5739 394301 1.7.2.1 nitrite reductase (NO-forming) mitochondrion - 5739 742855 1.7.2.1 nitrite reductase (NO-forming) mitochondrion the first 65 amino acid residues of theN-terminal domain constitute a mitochondrial targeting signal, sequence analysis 5739 695769 1.7.2.2 nitrite reductase (cytochrome; ammonia-forming) mitochondrion - 5739 742913 1.7.2.5 nitric oxide reductase (cytochrome c) mitochondrion - 5739 -, 395119, 688826 1.7.3.3 factor-independent urate hydroxylase mitochondrion - 5739 394164 1.8.1.11 asparagusate reductase mitochondrion - 5739 394702, 394703, 394704 1.8.1.4 dihydrolipoyl dehydrogenase mitochondrion - 5739 349067, 393986, 393991, 393994, 394002, 658249, 673517, 691036, 693975, 694356, 694854, 725073, 741807, 741998, 742087, 743051, 743052, 743450, 743767, 763920, 765736 1.8.1.4 dihydrolipoyl dehydrogenase mitochondrion DLDH exclusively exists in mitochondria 5739 692060 1.8.1.4 dihydrolipoyl dehydrogenase mitochondrion E3 is a mitochondrial protein that is synthesized in precursor form in the cytoplasm. The precursor E3 for humans has a leader sequence composed of 35-amino acids at its amino-terminus, which is cleaved off during its import into the mitochondria matrix 5739 743050 1.8.1.4 dihydrolipoyl dehydrogenase mitochondrion enzyme is part of multienzyme complexes in the inner mitochondrial membrane 5739 659866 1.8.1.4 dihydrolipoyl dehydrogenase mitochondrion in spermatocytes 5739 659482 1.8.1.4 dihydrolipoyl dehydrogenase mitochondrion isozyme mLipDH is a component of the branched-chain 2-ketoacid dehydrogenase and the 2-ketoglutarate dehydrogenase multienzyme complexes 5739 656835 1.8.1.4 dihydrolipoyl dehydrogenase mitochondrion Lpd1 protein is localized in the mitochondria during the logarithmic yeast growth and the filamentous growth 5739 -, 712087 1.8.1.4 dihydrolipoyl dehydrogenase mitochondrion mitochondrial matrix 5739 -, 711320 1.8.1.7 glutathione-disulfide reductase mitochondrion - 5739 -, 676198, 726192, 726208, 726543, 764549 1.8.1.7 glutathione-disulfide reductase mitochondrion cytoplasm-like spaces of mitochondria 5739 393993, 394708 1.8.1.7 glutathione-disulfide reductase mitochondrion intermembrane space and matrix 5739 660197 1.8.1.7 glutathione-disulfide reductase mitochondrion isoform GR2 5739 -, 701018, 741875 1.8.1.7 glutathione-disulfide reductase mitochondrion small amount 5739 394756 1.8.1.9 thioredoxin-disulfide reductase mitochondrion - 5739 394945, 394952, 658755, 658794, 671772, 676780, 684976, 685151, 685189, 688370, 689463, 696235, 700171, 710835, 712064, 712943, 743535, 743761, 764704, 765685 1.8.1.9 thioredoxin-disulfide reductase mitochondrion isoform TR2 5739 394945, 712944 1.8.1.9 thioredoxin-disulfide reductase mitochondrion isoform TR3 5739 394945 1.8.1.9 thioredoxin-disulfide reductase mitochondrion isoform TrxR2 5739 394954, 711101, 711478, 711755, 713184, 725059 1.8.1.9 thioredoxin-disulfide reductase mitochondrion isoform TrxR3 and TrxR1 5739 394949 1.8.1.9 thioredoxin-disulfide reductase mitochondrion isoform Txnrd2 5739 696435, 712446 1.8.1.9 thioredoxin-disulfide reductase mitochondrion TR3 5739 674618 1.8.1.9 thioredoxin-disulfide reductase mitochondrion TrxR-1(mito) 5739 394949 1.8.1.9 thioredoxin-disulfide reductase mitochondrion TrxR2 5739 674452 1.8.1.B1 thioredoxin glutathione reductase mitochondrion - 5739 690173, 725773 1.8.1.B1 thioredoxin glutathione reductase mitochondrion isoform mTGR 5739 742460 1.8.1.B1 thioredoxin glutathione reductase mitochondrion mitochondrial and cytosolic variants of TGR are generated from a single TGR gene 5739 687495 1.8.2.1 sulfite dehydrogenase (cytochrome) mitochondrion - 5739 711223 1.8.3.1 sulfite oxidase mitochondrion - 5739 392991, 392999, 393005, 393008, 393011, 393016, 694617, 701313, 741618, 764106, 765392, 765457 1.8.3.1 sulfite oxidase mitochondrion intermembrane space 5739 392996, 392997, 393007, 393012 1.8.3.1 sulfite oxidase mitochondrion mitochondrial intermembrane space 5739 764105 1.8.3.2 thiol oxidase mitochondrion - 5739 -, 659862, 691085, 704513, 742434, 765345 1.8.3.2 thiol oxidase mitochondrion Erv1 requires the redox-regulated receptor Mia40 for its import into mitochondria. Interacts via disulfide bonds with Mia40. It does not need two CX2C motifs for import into mitochondria 5739 692335 1.8.3.2 thiol oxidase mitochondrion in neurons 5739 668116 1.8.3.2 thiol oxidase mitochondrion intermembrane space 5739 393060, 658616 1.8.3.2 thiol oxidase mitochondrion preferentially 5739 659339 1.8.3.2 thiol oxidase mitochondrion protein Alr is required for mitochondrial biogenesis of human Mia40, which is responsible for the import and oxidative folding of proteins destined for the intermembrane space of mitochondria 5739 726522 1.8.4.11 peptide-methionine (S)-S-oxide reductase mitochondrion - 5739 -, 657754, 667679, 668092, 668513, 684984, 686598, 686891, 698124, 712073, 741498, 741832, 741930, 742409, 742555, 763918, 764710 1.8.4.11 peptide-methionine (S)-S-oxide reductase mitochondrion matrix 5739 658217 1.8.4.11 peptide-methionine (S)-S-oxide reductase mitochondrion matrix, mitochondrial isozyme of MsrA 5739 657828 1.8.4.11 peptide-methionine (S)-S-oxide reductase mitochondrion mitochondrial splicing variant MsrA 5739 668092 1.8.4.11 peptide-methionine (S)-S-oxide reductase mitochondrion MsrA contains a mitochondrial targeting sequence which is not necessary for catalytic activity 5739 394111 1.8.4.12 peptide-methionine (R)-S-oxide reductase mitochondrion - 5739 -, 657754, 686598, 687720, 696601, 710848, 711940, 726460, 763918, 764698, 764710 1.8.4.12 peptide-methionine (R)-S-oxide reductase mitochondrion isozyme MsrB2 or CBS-1, and isozyme MsrB3 5739 669008 1.8.4.12 peptide-methionine (R)-S-oxide reductase mitochondrion isozymes MsrB2 and MsrB3 5739 668357 1.8.4.12 peptide-methionine (R)-S-oxide reductase mitochondrion matrix 5739 658217 1.8.4.12 peptide-methionine (R)-S-oxide reductase mitochondrion MsrB3B contains a different signal peptide at the N-terminus and is targeted to mitochondria 5739 684984 1.8.4.12 peptide-methionine (R)-S-oxide reductase mitochondrion Sel-X, a MsrB enzyme form variant 5739 658212 1.8.4.12 peptide-methionine (R)-S-oxide reductase mitochondrion Sel-X, a MsrB enzyme form variant, sulindac reducing activity in calf 5739 658212 1.8.4.12 peptide-methionine (R)-S-oxide reductase mitochondrion Sel-X, a MsrB enzyme variant 5739 658212 1.8.4.12 peptide-methionine (R)-S-oxide reductase mitochondrion sequence contains a mitochondrial signal sequence 5739 -, 742044 1.8.4.12 peptide-methionine (R)-S-oxide reductase mitochondrion two splice variants of the enzyme (Msr B3) that differ in their N-terminal signal sequence, which directs the protein to either the endoplasmic reticulum (ER) or mitochondria 5739 764103 1.8.4.2 protein-disulfide reductase (glutathione) mitochondrion - 5739 763028 1.8.5.1 glutathione dehydrogenase (ascorbate) mitochondrion - 5739 -, 765156, 765592, 765624 1.8.5.4 bacterial sulfide:quinone reductase mitochondrion - 5739 -, 716170, 726049, 742246, 765750 1.8.5.8 eukaryotic sulfide quinone oxidoreductase mitochondrion - 5739 -, 744869, 745263, 745313, 745666, 745667, 764408, 764431 1.8.5.8 eukaryotic sulfide quinone oxidoreductase mitochondrion ScSQR is located only in the mitochondria, no signal peptide is found 5739 764525 1.8.98.2 sulfiredoxin mitochondrion - 5739 698993, 742566, 742996 1.8.98.2 sulfiredoxin mitochondrion dual localization to plastid and mitochondrion, in line with the prediction of a signal peptide for dual targeting. In mitochondria, enzyme interacts with peroxiredoxin IIF and thioredon. Sulfiredoxin catalyzes the retroreduction of the inactive sulfinic form of atypical Prx IIF using thioredoxin as reducing agent 5739 726220 1.8.98.2 sulfiredoxin mitochondrion dual localization to plastid and mitochondrion, in line with the prediction of a signal peptide for dual targeting. In mitochondria, enzyme interacts with peroxiredoxin IIF and thioredoxin. Sulfiredoxin catalyzes the retroreduction of the inactive sulfinic form of atypical Prx IIF using thioredoxin as reducing agent 5739 726220 1.97.1.4 [formate-C-acetyltransferase]-activating enzyme mitochondrion - 5739 674782 2.1.1.114 polyprenyldihydroxybenzoate methyltransferase mitochondrion - 5739 756974 2.1.1.114 polyprenyldihydroxybenzoate methyltransferase mitochondrion the Coq3 polypeptide is peripherally associated with the matrix side of the inner membrane of yeast mitochondria 5739 485140 2.1.1.13 methionine synthase mitochondrion - 5739 441183 2.1.1.13 methionine synthase mitochondrion 10% of total activity 5739 441216 2.1.1.168 21S rRNA (uridine2791-2'-O)-methyltransferase mitochondrion - 5739 697605 2.1.1.17 phosphatidylethanolamine N-methyltransferase mitochondrion - 5739 485182 2.1.1.17 phosphatidylethanolamine N-methyltransferase mitochondrion mitochondria associated membrane 5739 485179, 485182, 485192 2.1.1.201 2-methoxy-6-polyprenyl-1,4-benzoquinol methylase mitochondrion - 5739 704387, 704388, 706320, 756080, 756974 2.1.1.201 2-methoxy-6-polyprenyl-1,4-benzoquinol methylase mitochondrion the COQ5 polypeptide is associated with the mitochondrial inner membrane on the matrix side 5739 735704 2.1.1.203 tRNA (cytosine34-C5)-methyltransferase mitochondrion no obvious mitochondrial-targeting sequence is present in the N-terminal region of NSUN3, immunohistochemic analysis shows that NSUN3 predominantly localized in mitochondria 5739 757733 2.1.1.203 tRNA (cytosine34-C5)-methyltransferase mitochondrion NSUN3 has been shown to localize to the mitochondrial matrix, its mitochondrial import has not been determined 5739 756866 2.1.1.220 tRNA (adenine58-N1)-methyltransferase mitochondrion only subunit Trm61 5739 756160 2.1.1.222 2-polyprenyl-6-hydroxyphenol methylase mitochondrion the Coq3 polypeptide is peripherally associated with the matrix side of the inner membrane of yeast mitochondria 5739 485140 2.1.1.228 tRNA (guanine37-N1)-methyltransferase mitochondrion - 5739 735421 2.1.1.228 tRNA (guanine37-N1)-methyltransferase mitochondrion TbTRM5 localizes to nucleus and mitochondrion 5739 -, 737228 2.1.1.244 protein N-terminal methyltransferase mitochondrion - 5739 756463 2.1.1.33 tRNA (guanine46-N7)-methyltransferase mitochondrion - 5739 441471 2.1.1.373 2-hydroxy-4-(methylsulfanyl)butanoate S-methyltransferase mitochondrion - 5739 -, 759840 2.1.1.41 sterol 24-C-methyltransferase mitochondrion - 5739 485328 2.1.1.41 sterol 24-C-methyltransferase mitochondrion major activity 5739 485327 2.1.1.49 amine N-methyltransferase mitochondrion liver 5739 441356 2.1.1.56 mRNA (guanine-N7)-methyltransferase mitochondrion of infected BHK cells 5739 485451 2.1.1.61 tRNA 5-(aminomethyl)-2-thiouridylate-methyltransferase mitochondrion - 5739 658202, 701608, 757188, 757527, 758370 2.1.1.61 tRNA 5-(aminomethyl)-2-thiouridylate-methyltransferase mitochondrion localizes exclusively to mitochondria, even when overexpressed 5739 671744 2.1.1.64 3-demethylubiquinol 3-O-methyltransferase mitochondrion - 5739 485508 2.1.1.64 3-demethylubiquinol 3-O-methyltransferase mitochondrion the Coq3 polypeptide is peripherally associated with the matrix side of the inner membrane of yeast mitochondria 5739 485140 2.1.1.71 phosphatidyl-N-methylethanolamine N-methyltransferase mitochondrion - 5739 485193, 485555, 485560 2.1.1.77 protein-L-isoaspartate(D-aspartate) O-methyltransferase mitochondrion - 5739 756674 2.1.1.77 protein-L-isoaspartate(D-aspartate) O-methyltransferase mitochondrion isoforms PIMT2 TISIbetapsi, PIMT2 TISIalphapsi, and PIMT2 TISIalphaomega are predominately present in small, motile bodies suspected of being mitochondria 5739 706232 2.1.1.B123 16S rRNA (uridine1369-2'-O)-methyltransferase mitochondrion - 5739 757633 2.1.1.B124 16S rRNA (guanine1370-2'-O)-methyltransferase mitochondrion - 5739 757147, 757634 2.1.1.B125 16S rRNA (guanine1145-2'-O)-methyltransferase mitochondrion - 5739 757147 2.1.2.1 glycine hydroxymethyltransferase mitochondrion - 5739 -, 391984, 441391, 441394, 441398, 441399, 441401, 441405, 441419, 441420, 441427, 441437, 662384, 676503, 700740, 703863, 720740, 736882, 756725, 757362 2.1.2.1 glycine hydroxymethyltransferase mitochondrion 5% of the enzyme activity 5739 441403 2.1.2.1 glycine hydroxymethyltransferase mitochondrion from rat liver fed dietary pyridoxine ranging from adequate to deficient levels, 2-0 mg pyridoxine per kg diet. SHMT activity increases with increasing dietary pyridoxine concentrations. The mitochondrial isoenzyme comprises about 70% of the total activity 5739 659772 2.1.2.1 glycine hydroxymethyltransferase mitochondrion isoform SHMT2 5739 756058, 756098, 756695 2.1.2.1 glycine hydroxymethyltransferase mitochondrion isozyme PfSHMTc in a stage-specific manner. Isozyme PfSHMTm showa a distinctly more pronounced mitochondrial location through most of the erythrocytic cycle, presence of a mitochondrial signal sequence 5739 720362 2.1.2.1 glycine hydroxymethyltransferase mitochondrion matrix 5739 441420 2.1.2.1 glycine hydroxymethyltransferase mitochondrion mitochondrial isoform SHMT2 5739 736925 2.1.2.1 glycine hydroxymethyltransferase mitochondrion mitochondrial isozyme SHMT2 5739 736727 2.1.2.1 glycine hydroxymethyltransferase mitochondrion peptide sequence alignment with the known SHMT from other species and the prospective zebrafish cytosolic SHMT as well as signal prediction software reveals a potential mitochondrial signal peptide cleavage site between residues 20 and 30 5739 686353 2.1.2.1 glycine hydroxymethyltransferase mitochondrion SHMT2 encodes the mitochondrial isozyme SHMT2 and the cytoplasmic/nuclear isozyme SHMT2alpha through alternative promoter uses 5739 720054 2.1.2.10 aminomethyltransferase mitochondrion - 5739 485693, 485696, 485697, 705607, 706478 2.1.2.3 phosphoribosylaminoimidazolecarboxamide formyltransferase mitochondrion - 5739 485745 2.1.2.9 methionyl-tRNA formyltransferase mitochondrion - 5739 -, 485803, 485807, 485808, 485813, 673867, 704594, 719239, 736461, 757809 2.1.3.3 ornithine carbamoyltransferase mitochondrion - 5739 485917, 485922, 673136, 720095 2.1.3.3 ornithine carbamoyltransferase mitochondrion 2 enzyme forms in sugar cane: cytoplasmic and mitochondrial 5739 485895 2.1.3.3 ornithine carbamoyltransferase mitochondrion soluble matrix 5739 485901 2.1.3.8 lysine carbamoyltransferase mitochondrion - 5739 485958 2.1.3.8 lysine carbamoyltransferase mitochondrion localized outside the inner mitochondrial membrane 5739 485959 2.1.4.1 glycine amidinotransferase mitochondrion - 5739 -, 485967, 485971, 485973, 485974, 485975, 485978, 735972, 737153 2.2.1.2 transaldolase mitochondrion - 5739 486039 2.2.1.5 2-hydroxy-3-oxoadipate synthase mitochondrion - 5739 395925, 395926, 395930, 395933, 395935 2.2.1.5 2-hydroxy-3-oxoadipate synthase mitochondrion exclusively 5739 395924 2.2.1.5 2-hydroxy-3-oxoadipate synthase mitochondrion part of alpha-ketoglutarate dehydrogenase complex 5739 349030, 395924, 395936 2.2.1.6 acetolactate synthase mitochondrion - 5739 -, 33969, 395883, 395890, 395896, 718641, 734734 2.2.1.6 acetolactate synthase mitochondrion an N-terminal peptide, which is subsequently removed, is required to direct the protein to mitochondria in fungi 5739 700782 2.2.1.6 acetolactate synthase mitochondrion MoIlv2 and MoIlv6 are both localized to the mitochondria and the signal peptide of MoIlv6 is critical for its localization 5739 -, 734734 2.3.1.1 amino-acid N-acetyltransferase mitochondrion - 5739 661264, 676019, 737116, 758389 2.3.1.1 amino-acid N-acetyltransferase mitochondrion the enzyme contains an N-terminal putative mitochondrial targeting sequence 5739 662881 2.3.1.12 dihydrolipoyllysine-residue acetyltransferase mitochondrion - 5739 348910, 348951, 348955, 486196, 486238, 642164, 663011, 735426, 94893 2.3.1.12 dihydrolipoyllysine-residue acetyltransferase mitochondrion pyruvate dehydrogenase complex bound to inner mitochondrial membrane 5739 348910 2.3.1.127 ornithine N-benzoyltransferase mitochondrion renal and hepatic 5739 486248 2.3.1.129 acyl-[acyl-carrier-protein]-UDP-N-acetylglucosamine O-acyltransferase mitochondrion - 5739 720913 2.3.1.13 glycine N-acyltransferase mitochondrion - 5739 486265, 486266, 486268, 486269, 718757, 719351 2.3.1.13 glycine N-acyltransferase mitochondrion kidney enzyme is located in the matrix, probably not membrane bound 5739 486267 2.3.1.137 carnitine O-octanoyltransferase mitochondrion - 5739 -, 486290, 486291, 486293, 486294, 486295, 486296, 486297, 486298, 486299, 486300, 486303, 486309, 657546, 659372, 686028 2.3.1.137 carnitine O-octanoyltransferase mitochondrion innermitochondrial membrane 5739 -, 486291 2.3.1.137 carnitine O-octanoyltransferase mitochondrion synaptic and non-synaptic mitochondria 5739 -, 486296 2.3.1.137 carnitine O-octanoyltransferase mitochondrion the crat gene contains 2 alternative start codons, translation from the first codon results in a protein of 626 amino acids, with a 21-amino acid leader sequence that targets the CRAT protein to mitochondria 5739 -, 686028 2.3.1.14 glutamine N-phenylacetyltransferase mitochondrion - 5739 486317 2.3.1.14 glutamine N-phenylacetyltransferase mitochondrion inner membrane and matrix 5739 486318 2.3.1.15 glycerol-3-phosphate 1-O-acyltransferase mitochondrion - 5739 -, 486336, 486342, 486346, 486348, 486350, 486352, 486353, 486375, 486376, 486380, 486382, 486385, 486386, 486390, 671231, 673514, 674454, 684683, 684701, 684909, 685343, 686601, 688229, 701623, 702011, 735705, 736424, 736720, 748445, 757543 2.3.1.15 glycerol-3-phosphate 1-O-acyltransferase mitochondrion 2 isozymes in mitochondria, mitochondrial isozymes mtGPAT1 and mtGPAT2 5739 662192 2.3.1.15 glycerol-3-phosphate 1-O-acyltransferase mitochondrion activity with palmitoyl-CoA is reduced in mitochondria of aged livers 5739 661226 2.3.1.15 glycerol-3-phosphate 1-O-acyltransferase mitochondrion DTNB-resistant isozyme 5739 486347 2.3.1.15 glycerol-3-phosphate 1-O-acyltransferase mitochondrion heart 5739 685377 2.3.1.15 glycerol-3-phosphate 1-O-acyltransferase mitochondrion higly active in mitochondria 5739 705466 2.3.1.15 glycerol-3-phosphate 1-O-acyltransferase mitochondrion intermembrane space 5739 486341 2.3.1.15 glycerol-3-phosphate 1-O-acyltransferase mitochondrion isoforms mtGPAT1 and mtGPAT2 5739 672373 2.3.1.15 glycerol-3-phosphate 1-O-acyltransferase mitochondrion mitochondrial isozyme 5739 660676 2.3.1.16 acetyl-CoA C-acyltransferase mitochondrion - 5739 288678, 288681, 486403, 486404, 486409, 486422, 660434, 664215, 735368, 756086 2.3.1.16 acetyl-CoA C-acyltransferase mitochondrion binding to inner membrane 5739 33713 2.3.1.168 dihydrolipoyllysine-residue (2-methylpropanoyl)transferase mitochondrion - 5739 644840, 644843, 644845, 644846, 644848 2.3.1.17 aspartate N-acetyltransferase mitochondrion - 5739 658360, 659757 2.3.1.17 aspartate N-acetyltransferase mitochondrion enzyme is present in both cytoplasm and mitochondria 5739 719199 2.3.1.174 3-oxoadipyl-CoA thiolase mitochondrion - 5739 -, 756853 2.3.1.181 lipoyl(octanoyl) transferase mitochondrion - 5739 662098, 663062, 688368, 737026 2.3.1.191 UDP-3-O-(3-hydroxyacyl)glucosamine N-acyltransferase mitochondrion LpxD proteins in Arabidopsis thaliana are targeted to the mitochondria, subcellular localization analysis, overview 5739 -, 720913 2.3.1.192 glycine N-phenylacetyltransferase mitochondrion - 5739 487438, 487441, 707446, 708944 2.3.1.20 diacylglycerol O-acyltransferase mitochondrion - 5739 486483, 719951 2.3.1.21 carnitine O-palmitoyltransferase mitochondrion - 5739 -, 486522, 486523, 486526, 486527, 486528, 486529, 486530, 486531, 486532, 486535, 486536, 486537, 486538, 486539, 486542, 486543, 486544, 486545, 486546, 486548, 486551, 486552, 486553, 486555, 486556, 486557, 486558, 486559, 486561, 486562, 486563, 486564, 486566, 486567, 486568, 486570, 486573, 486580, 486589, 487413, 657733, 659245, 659306, 672147, 672836, 673451, 674520, 674818, 675569, 675571, 675797, 686125, 688449, 702111, 702112, 702130, 703293, 703383, 705679, 720444, 735697, 736077, 736129, 736183, 736717, 756509, 757297 2.3.1.21 carnitine O-palmitoyltransferase mitochondrion 2 isoforms of CPT I, L-CPT I and M-CPT I 5739 486571, 486577, 486582, 486585, 486591 2.3.1.21 carnitine O-palmitoyltransferase mitochondrion CPT I 5739 486560, 486572, 486576, 486578 2.3.1.21 carnitine O-palmitoyltransferase mitochondrion CPT II 5739 486541, 486576 2.3.1.21 carnitine O-palmitoyltransferase mitochondrion mitochondrial outer membrane, enzyme is found in large molecular mass complexes that also contain long chain acyl-CoA synthetase and the voltage-dependent anion channel, with strong interaction between the three proteins 5739 719944 2.3.1.21 carnitine O-palmitoyltransferase mitochondrion of newborn, 24-h old, fed or fasted, and 5-months-old pigs 5739 718550 2.3.1.21 carnitine O-palmitoyltransferase mitochondrion outer membrane 5739 659393 2.3.1.21 carnitine O-palmitoyltransferase mitochondrion recombinant L-CPT I and M-CPT I in Pichia pastoris 5739 486585 2.3.1.23 1-acylglycerophosphocholine O-acyltransferase mitochondrion - 5739 486621, 486629, 657864 2.3.1.23 1-acylglycerophosphocholine O-acyltransferase mitochondrion probably associated with the outer membrane 5739 486647 2.3.1.234 N6-L-threonylcarbamoyladenine synthase mitochondrion Sua5 catalyzes the first step leading to the threonyl-carbamoyl-AMP intermediate. Proteins Qri7 and Sua5 together constitute the mitochondrial pathway for the biosynthesis of N6-threonylcarbamoyladenosine. The import of cytoplasmic Sua5 into the mitochondria is required for this organelle to be functional 5739 736030 2.3.1.25 plasmalogen synthase mitochondrion - 5739 486649 2.3.1.26 sterol O-acyltransferase mitochondrion little activity 5739 -, 486685 2.3.1.268 ethanol O-acetyltransferase mitochondrion - 5739 -, 755853 2.3.1.268 ethanol O-acetyltransferase mitochondrion the N-terminal domain of enzyme Eat1 is responsible for targeting 5739 -, 755709 2.3.1.271 L-glutamate-5-semialdehyde N-acetyltransferase mitochondrion - 5739 -, 747802 2.3.1.286 protein acetyllysine N-acetyltransferase mitochondrion - 5739 738108, 738661 2.3.1.286 protein acetyllysine N-acetyltransferase mitochondrion SIRT3 is localized to the inner membrane of the mitochondria 5739 730840 2.3.1.286 protein acetyllysine N-acetyltransferase mitochondrion Sirt5 can be translocated into the mitochondrial intermembrane space, but also into the matrix 5739 730205 2.3.1.29 glycine C-acetyltransferase mitochondrion - 5739 486744, 486745, 486746, 486747 2.3.1.29 glycine C-acetyltransferase mitochondrion enzyme imported into mitochondrion from cytoplasm 5739 486747 2.3.1.30 serine O-acetyltransferase mitochondrion - 5739 486775, 660267, 671439, 689475, 689626 2.3.1.30 serine O-acetyltransferase mitochondrion 76% of total activity 5739 486764 2.3.1.30 serine O-acetyltransferase mitochondrion between one-third and two-third of the activity 5739 486754 2.3.1.30 serine O-acetyltransferase mitochondrion compartment with largest serine acetyltransferase activity of total serine acetyltransferase activity independent of cytosolic serine acetyltransferase activity 5739 706161 2.3.1.30 serine O-acetyltransferase mitochondrion compartment with largest serine acetyltransferase activity of total serine acetyltransferase activity independent of plastidic serine acetyltransferase 5739 706161 2.3.1.30 serine O-acetyltransferase mitochondrion isozyme SAT-m, matrix 5739 486765, 486775 2.3.1.30 serine O-acetyltransferase mitochondrion isozyme VvSERAT2-2, mainly 5739 736161 2.3.1.30 serine O-acetyltransferase mitochondrion mitochondrial SATase Serat2,2 5739 671276 2.3.1.37 5-aminolevulinate synthase mitochondrion - 5739 486814, 486818, 486820, 486822, 486823, 486827, 486833, 486835, 486836, 486838, 486841, 486843, 486850, 486860, 661895, 719402, 735684, 737145, 756863, 757560, 758491 2.3.1.37 5-aminolevulinate synthase mitochondrion major activity 5739 486816 2.3.1.39 [acyl-carrier-protein] S-malonyltransferase mitochondrion - 5739 703315, 756900, 757892, 757986 2.3.1.39 [acyl-carrier-protein] S-malonyltransferase mitochondrion enzyme is part of the malonyl-CoA-dependent type II fatty acid synthase complex in mitochondria, and contains a mitochondrial targeting sequence 5739 659451 2.3.1.41 beta-ketoacyl-[acyl-carrier-protein] synthase I mitochondrion - 5739 659442, 659451 2.3.1.41 beta-ketoacyl-[acyl-carrier-protein] synthase I mitochondrion beta-ketoacyl [ACP] synthase from the mitochondrial type II fatty acid synthase 5739 677030 2.3.1.41 beta-ketoacyl-[acyl-carrier-protein] synthase I mitochondrion enzyme in the elongase complex 5739 674882 2.3.1.42 glycerone-phosphate O-acyltransferase mitochondrion - 5739 486950, 486952, 486969 2.3.1.42 glycerone-phosphate O-acyltransferase mitochondrion membrane-bound, may be side-reaction of glycerol 3-phosphate acyltransferase 5739 486962 2.3.1.42 glycerone-phosphate O-acyltransferase mitochondrion outer membrane 5739 486338 2.3.1.48 histone acetyltransferase mitochondrion - 5739 -, 755921, 757681 2.3.1.51 1-acylglycerol-3-phosphate O-acyltransferase mitochondrion - 5739 486341, 720178, 735703, 735714, 756108 2.3.1.51 1-acylglycerol-3-phosphate O-acyltransferase mitochondrion the presence of the enzyme in the mitochondrial fraction cannot be explained by the contamination of microsomes in this fraction 5739 -, 486375 2.3.1.54 formate C-acetyltransferase mitochondrion - 5739 674782 2.3.1.57 diamine N-acetyltransferase mitochondrion - 5739 -, 487256 2.3.1.57 diamine N-acetyltransferase mitochondrion mainly 5739 703097 2.3.1.57 diamine N-acetyltransferase mitochondrion mitochondrial uptake may be regulated by the phosphorylation state of SSAT 5739 701622 2.3.1.61 dihydrolipoyllysine-residue succinyltransferase mitochondrion - 5739 -, 349039, 487341, 487344, 487345, 659354, 703808, 705900, 735521 2.3.1.61 dihydrolipoyllysine-residue succinyltransferase mitochondrion matrix 5739 676757 2.3.1.61 dihydrolipoyllysine-residue succinyltransferase mitochondrion the enzyme localizes to the mitochondrial matrix 5739 735580 2.3.1.68 glutamine N-acyltransferase mitochondrion - 5739 486266 2.3.1.7 carnitine O-acetyltransferase mitochondrion - 5739 -, 486295, 487400, 487403, 487404, 487406, 487407, 487408, 487410, 487411, 487413, 487414, 487415, 487417, 487418, 487421, 487422, 487423, 487424, 487425, 487426, 487427, 487428, 487430, 487431, 487432, 686262, 735697, 735852, 736607 2.3.1.7 carnitine O-acetyltransferase mitochondrion isoform Cat2 5739 719875 2.3.1.7 carnitine O-acetyltransferase mitochondrion membrane-bound 5739 -, 487400, 487416, 487421, 487422, 487423, 487429 2.3.1.7 carnitine O-acetyltransferase mitochondrion sequence contains a N-terminal mitochondrial targeting sequence and a C-terminal peroxisomal targeting sequence and protein is localized to both peroxisomes and mitochondria 5739 719381 2.3.1.7 carnitine O-acetyltransferase mitochondrion the majority of Crat mRNA in mouse is mitochondrial, with only about 1–20% of Crat mRNA being peroxisomal, except for liver, where peroxisomal Crat represents about 38%of total Crat mRNA 5739 -, 686028 2.3.1.71 glycine N-benzoyltransferase mitochondrion - 5739 486265, 486269, 487437, 487438, 487440, 487441, 487442, 718757, 719351 2.3.1.71 glycine N-benzoyltransferase mitochondrion enzyme activity shows significant increase between the 1st and 7th day after common bile duct ligation 5739 487443 2.3.1.85 fatty-acid synthase system mitochondrion - 5739 703756, 704721, 705747, 706445 2.3.1.86 fatty-acyl-CoA synthase system mitochondrion - 5739 662160 2.3.1.9 acetyl-CoA C-acetyltransferase mitochondrion - 5739 -, 676035, 676336, 685147, 686989, 689028, 706822, 736771, 755863 2.3.1.9 acetyl-CoA C-acetyltransferase mitochondrion isoform Acat1 5739 -, 736168 2.3.1.97 glycylpeptide N-tetradecanoyltransferase mitochondrion - 5739 487773 2.3.1.97 glycylpeptide N-tetradecanoyltransferase mitochondrion occasionally 5739 487768 2.3.1.B41 protein-long-chain fatty-acyl-lysine deacylase (NAD+) mitochondrion - 5739 -, 756973 2.3.1.B43 protein-lysine desuccinylase (NAD+) mitochondrion - 5739 -, 729556, 729732, 730922, 731010, 755788, 756010, 756393, 756563, 756680, 757084, 757175, 757184, 757350, 757400, 758247 2.3.1.B43 protein-lysine desuccinylase (NAD+) mitochondrion primarily 5739 756555 2.3.1.B43 protein-lysine desuccinylase (NAD+) mitochondrion Sirt5 can be translocated into the mitochondrial intermembrane space, but also into the matrix 5739 730205 2.3.1.B43 protein-lysine desuccinylase (NAD+) mitochondrion SIRT5 is present both mitochondrially and extramitochondrially 5739 730390 2.3.1.B43 protein-lysine desuccinylase (NAD+) mitochondrion SIRT5 isoforms (i.e., SIRT5iso1 and SIRT5iso2) are encoded by the human SIRT5 gene. Their C-termini slightly differ from each other. Although both isoforms contain cleavable mitochondrial targeting signals at their N-termini, it is found that the cleaved SIRT5iso2 is localized mainly in mitochondria, whereas the cleaved SIRT5iso1 is localized in both mitochondria and cytoplasm. The C-terminus of SIRT5iso2, which is rich in hydrophobic amino acid residues, functions as a mitochondrial membrane insertion signal 5739 729801 2.3.1.B43 protein-lysine desuccinylase (NAD+) mitochondrion SIRT5 isozymes 1, 2 and 3 are mitochondria-localized. The full-length SIRT5 protein (SIRT5iso1) contains the N-terminal 36 amino acid mitochondrial localization signal (MLS) peptide 5739 755970 2.3.2.13 protein-glutamine gamma-glutamyltransferase mitochondrion - 5739 735910 2.3.2.13 protein-glutamine gamma-glutamyltransferase mitochondrion outer mitochondrial membrane of cells overexpressing enzyme 5739 659430 2.3.2.26 HECT-type E3 ubiquitin transferase mitochondrion mitochondrion-associated protein 5739 757186 2.3.2.27 RING-type E3 ubiquitin transferase mitochondrion - 5739 736504, 755737 2.3.2.31 RBR-type E3 ubiquitin transferase mitochondrion mitophagy is regulated by a positive ubiquitylation feedback loop in which the PINK1 kinase phosphorylates both ubiquitin and the E3 ubiquitin ligase parkin. This event recruits parkin to the mitochondria 5739 757005 2.3.2.5 glutaminyl-peptide cyclotransferase mitochondrion isoform isoDromeQC 5739 718944 2.3.2.B11 ubiquitin transferase RING E3 (calmodulin-selective) mitochondrion mitochondrion-associated protein 5739 757186 2.3.3.1 citrate (Si)-synthase mitochondrion - 5739 488073, 488083, 702966, 704788, 757468 2.3.3.1 citrate (Si)-synthase mitochondrion encoded by nuclear DNA, precursor is synthesized in the cytosol, import into mitochondrion 5739 488058 2.3.3.1 citrate (Si)-synthase mitochondrion mitochondrial adaptation 5739 488065 2.3.3.10 hydroxymethylglutaryl-CoA synthase mitochondrion - 5739 -, 488093, 488095, 488096, 488099, 488100, 488101, 488102, 488103, 488104, 488105, 488106, 488107, 488111, 488112, 488114, 488115, 488120, 488122, 657803, 706004, 706833, 726092 2.3.3.10 hydroxymethylglutaryl-CoA synthase mitochondrion distinct mitochondrial and cytoplasmic forms 5739 488099, 488101 2.3.3.10 hydroxymethylglutaryl-CoA synthase mitochondrion isoform HMGCS2 5739 720239 2.3.3.10 hydroxymethylglutaryl-CoA synthase mitochondrion ketogenic isoform 5739 488117 2.3.3.13 2-isopropylmalate synthase mitochondrion - 5739 -, 736032 2.3.3.13 2-isopropylmalate synthase mitochondrion alpha-isopropylmalate synthase Ia, MW 68000, is imported into the mitochondrial matrix 5739 636532 2.3.3.13 2-isopropylmalate synthase mitochondrion large enzyme form, matrix 5739 -, 636511 2.3.3.13 2-isopropylmalate synthase mitochondrion the Leu4 gene encodes 2 forms: a short cytoplasmic form and a long form that is targeted to the mitochondria 5739 636507 2.3.3.14 homocitrate synthase mitochondrion 25% of total activity 5739 488145 2.3.3.16 citrate synthase (unknown stereospecificity) mitochondrion - 5739 -, 488039, 488047, 488053, 488054, 488063, 488068, 488081, 656929, 686703, 705651, 706384, 720215, 736278, 736555, 736845, 737011, 755738, 756609, 757999 2.3.3.16 citrate synthase (unknown stereospecificity) mitochondrion citrate uptake into mitochondria 5739 488061 2.3.3.16 citrate synthase (unknown stereospecificity) mitochondrion mild reductions in mitochondrial citrate synthase activity results in a compromised nitrate assimilation and reduced leaf pigmentation but have no effect on photosynthetic performance or growth 5739 -, 689614 2.3.3.16 citrate synthase (unknown stereospecificity) mitochondrion mitochondrial isozyme 5739 488072, 488086 2.3.3.16 citrate synthase (unknown stereospecificity) mitochondrion N-terminal mitochondrial import signal sequence 5739 -, 488074 2.3.3.5 2-methylcitrate synthase mitochondrion - 5739 -, 5344, 735795 2.3.3.5 2-methylcitrate synthase mitochondrion enzyme possesses a mitochondrial signal import sequence 5739 488089, 661700 2.3.3.8 ATP citrate synthase mitochondrion the enzyme tends to associate with the mitochondria 5739 488195, 488196 2.4.1.123 inositol 3-alpha-galactosyltransferase mitochondrion isozyme BnGolS3-2 5739 759361 2.4.1.13 sucrose synthase mitochondrion - 5739 674564 2.4.1.13 sucrose synthase mitochondrion associated with mitochondrion 5739 720790 2.4.1.13 sucrose synthase mitochondrion at about 12 h of sucrose provision 5739 720323 2.4.1.17 glucuronosyltransferase mitochondrion - 5739 488753 2.4.1.173 sterol 3beta-glucosyltransferase mitochondrion - 5739 637775 2.4.1.182 lipid-A-disaccharide synthase mitochondrion - 5739 -, 718879 2.4.1.255 protein O-GlcNAc transferase mitochondrion - 5739 659497, 703882, 723585, 759487 2.4.1.255 protein O-GlcNAc transferase mitochondrion isoform 2 5739 735642 2.4.1.255 protein O-GlcNAc transferase mitochondrion mitochondrial and nuclear/cytoplasmic variants of OGT 5739 701899 2.4.1.62 ganglioside galactosyltransferase mitochondrion in fetal and adult brain 5739 -, 489307 2.4.1.79 globotriaosylceramide 3-beta-N-acetylgalactosaminyltransferase mitochondrion - 5739 489381 2.4.1.83 dolichyl-phosphate beta-D-mannosyltransferase mitochondrion cytosolic face of the outer membrane 5739 489433 2.4.1.83 dolichyl-phosphate beta-D-mannosyltransferase mitochondrion outer membrane 5739 489434, 489438 2.4.1.92 (N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase mitochondrion - 5739 489574 2.4.2.12 nicotinamide phosphoribosyltransferase mitochondrion - 5739 661421 2.4.2.4 thymidine phosphorylase mitochondrion neutrophil in gastric cancer tissue 5739 659750 2.4.2.59 sulfide-dependent adenosine diphosphate thiazole synthase mitochondrion - 5739 -, 744796 2.4.2.64 tRNA-guanosine34 queuine transglycosylase mitochondrion - 5739 759465 2.4.99.20 2'-phospho-ADP-ribosyl cyclase/2'-phospho-cyclic-ADP-ribose transferase mitochondrion 0.2 nM/min*mg 5739 724210 2.5.1.1 dimethylallyltranstransferase mitochondrion - 5739 739121 2.5.1.10 (2E,6E)-farnesyl diphosphate synthase mitochondrion - 5739 737749 2.5.1.15 dihydropteroate synthase mitochondrion - 5739 639674, 639684, 674784 2.5.1.17 corrinoid adenosyltransferase mitochondrion - 5739 674277, 676023 2.5.1.17 corrinoid adenosyltransferase mitochondrion Arg19 in the mitochondrial targeting sequence is important 5739 672110 2.5.1.17 corrinoid adenosyltransferase mitochondrion enzyme contains a putative mitochondrial targeting sequence 5739 659411 2.5.1.18 glutathione transferase mitochondrion - 5739 637932, 637938, 738384 2.5.1.29 geranylgeranyl diphosphate synthase mitochondrion - 5739 687623 2.5.1.39 4-hydroxybenzoate polyprenyltransferase mitochondrion - 5739 -, 636998, 636999, 637005, 657846, 671299, 674978 2.5.1.39 4-hydroxybenzoate polyprenyltransferase mitochondrion activity mainly detected in mitochondria 5739 705592 2.5.1.39 4-hydroxybenzoate polyprenyltransferase mitochondrion enzyme contains a mitochondrion targeting sequence 5739 705592 2.5.1.39 4-hydroxybenzoate polyprenyltransferase mitochondrion enzyme contains an N-terminal mitochondrial targeting sequence 5739 660199 2.5.1.39 4-hydroxybenzoate polyprenyltransferase mitochondrion low activity 5739 636997, 704366 2.5.1.39 4-hydroxybenzoate polyprenyltransferase mitochondrion mainly localized in the mitochondria 5739 759945 2.5.1.43 nicotianamine synthase mitochondrion the sequence contains a mitochondrial targeting peptide 5739 -, 738308 2.5.1.47 cysteine synthase mitochondrion - 5739 688427, 723782 2.5.1.47 cysteine synthase mitochondrion all isoenzymes 5739 637370 2.5.1.47 cysteine synthase mitochondrion isoenzyme C 5739 637387 2.5.1.47 cysteine synthase mitochondrion isoform C 5739 662538 2.5.1.47 cysteine synthase mitochondrion isoform OAS-TL C 5739 689472 2.5.1.47 cysteine synthase mitochondrion isozyme OAS-TL C 5739 739348, 739364 2.5.1.47 cysteine synthase mitochondrion serine O-acetyltransferase SAT3 and O-acetylserine sulfhydrolase can interact in plant mitochondria to form the cysteine synthase complex. Formation of the mitochondrial cysteine synthase complex might be promoted by the stabilizing effect of sulfide, by efficient export of O-acetylserine from mitochondria, or by a combination of both 5739 722770 2.5.1.74 1,4-dihydroxy-2-naphthoate polyprenyltransferase mitochondrion - 5739 737563 2.5.1.75 tRNA dimethylallyltransferase mitochondrion - 5739 489966 2.5.1.75 tRNA dimethylallyltransferase mitochondrion Trit1 encodes an amino-terminal mitochondrial targeting sequence that directs mitochondrial import and modification of mitochondrial-tRNAs 5739 760010 2.5.1.81 geranylfarnesyl diphosphate synthase mitochondrion - 5739 -, 739320 2.5.1.83 hexaprenyl diphosphate synthase [(2E,6E)-farnesyl-diphosphate specific] mitochondrion - 5739 -, 759355 2.5.1.84 all-trans-nonaprenyl diphosphate synthase [geranyl-diphosphate specific] mitochondrion mitchondrial isozyme OsSPS1 5739 739259 2.5.1.85 all-trans-nonaprenyl diphosphate synthase [geranylgeranyl-diphosphate specific] mitochondrion sole localization 5739 723133 2.5.1.91 all-trans-decaprenyl-diphosphate synthase mitochondrion - 5739 707348 2.5.1.92 (2Z,6Z)-farnesyl diphosphate synthase mitochondrion probably 5739 -, 723434 2.5.1.B14 (2E,6E)-farnesyl-diphosphate all-trans-nonaprenyl diphosphate synthase mitochondrion - 5739 704891, 759800 2.5.1.B14 (2E,6E)-farnesyl-diphosphate all-trans-nonaprenyl diphosphate synthase mitochondrion isoform SPS1 5739 759800 2.5.1.B14 (2E,6E)-farnesyl-diphosphate all-trans-nonaprenyl diphosphate synthase mitochondrion isoform SPS1 shows a putative mitochondrial signal peptide sequence 5739 704891 2.5.1.B14 (2E,6E)-farnesyl-diphosphate all-trans-nonaprenyl diphosphate synthase mitochondrion mitchondrial isozyme OsSPS1 5739 739259 2.6.1.1 aspartate transaminase mitochondrion - 5739 639821, 639829, 639834, 639836, 639846, 639850, 660009, 671602, 672283, 675514, 676157, 676511, 737887, 738861, 759921 2.6.1.1 aspartate transaminase mitochondrion 2.8% activity is localized in mitochondria 5739 -, 672467 2.6.1.1 aspartate transaminase mitochondrion enzyme is constitutively expressed 5739 688897 2.6.1.1 aspartate transaminase mitochondrion enzyme is down-regulated in bloddstream forms of Trypanosoma brucei 5739 688897 2.6.1.1 aspartate transaminase mitochondrion form I and II occurs in chloroplast-enriched fraction 5739 639862 2.6.1.1 aspartate transaminase mitochondrion form II and III in poorly resolved plastid and mitochondrial fraction 5739 639854 2.6.1.1 aspartate transaminase mitochondrion isoenzyme AAT-2: major isoenzyme in mitochondria and peroxisomes 5739 639819 2.6.1.1 aspartate transaminase mitochondrion isozyme AAT1 5739 639876 2.6.1.1 aspartate transaminase mitochondrion isozyme B 5739 639833 2.6.1.1 aspartate transaminase mitochondrion mature isozyme mAAT 5739 639886 2.6.1.1 aspartate transaminase mitochondrion mature mAspAT and premature pmAspAT forms of the isozyme 5739 639875, 639880 2.6.1.1 aspartate transaminase mitochondrion mitochondrial isoenzyme 5739 636716, 639817, 639822, 639824, 639828, 639831, 639832, 639838, 639839, 639846, 639854, 639865, 639866, 639872, 639878, 639886 2.6.1.1 aspartate transaminase mitochondrion multiple catalytically active forms of mitochondrial isozyme 5739 639865, 639867 2.6.1.117 L-glutamine-4-(methylsulfanyl)-2-oxobutanoate aminotransferase mitochondrion - 5739 755020 2.6.1.120 beta-alanine—2-oxoglutarate transaminase mitochondrion - 5739 763027 2.6.1.120 beta-alanine—2-oxoglutarate transaminase mitochondrion mitochondrial matrix 5739 763379 2.6.1.13 ornithine aminotransferase mitochondrion - 5739 637117, 637126, 637136, 637137, 671263, 676887, 685833, 703993, 706356, 758976, 759181, 759545, 760090 2.6.1.13 ornithine aminotransferase mitochondrion colocalization of enzyme and arginase II within mitochondria of proximal straight tubule 5739 660684 2.6.1.13 ornithine aminotransferase mitochondrion the mitochondrial localization may control the reaction kinetics, OAT activity being apparently limited by the rate of ornithine entry into the mitochondrion 5739 758876 2.6.1.14 asparagine-oxo-acid transaminase mitochondrion - 5739 637254, 637255 2.6.1.15 glutamine-pyruvate transaminase mitochondrion - 5739 636642, 639897 2.6.1.18 beta-alanine-pyruvate transaminase mitochondrion the first 93 nucleotides of the full-length cDNA are predicted to encode the signal sequence that denote localization to the mitochondria 5739 -, 759951 2.6.1.19 4-aminobutyrate-2-oxoglutarate transaminase mitochondrion - 5739 -, 637032, 637034, 637037, 637038, 637041, 637042, 637045, 637046, 637058, 637060, 637079, 700887, 704874, 723611, 738002, 758692, 759407 2.6.1.19 4-aminobutyrate-2-oxoglutarate transaminase mitochondrion isoform GABA-T1 5739 704882 2.6.1.2 alanine transaminase mitochondrion - 5739 636715, 636721, 675140, 676963, 723400, 739457, 759663, 760005 2.6.1.22 (S)-3-amino-2-methylpropionate transaminase mitochondrion - 5739 636964 2.6.1.24 diiodotyrosine transaminase mitochondrion - 5739 639936 2.6.1.39 2-aminoadipate transaminase mitochondrion - 5739 -, 636620, 636622, 636623, 639979, 703133 2.6.1.39 2-aminoadipate transaminase mitochondrion glutamate-alpha-ketoadipate transaminase I 5739 639974 2.6.1.39 2-aminoadipate transaminase mitochondrion isoenzyme AadAT-II 5739 639983 2.6.1.40 (R)-3-amino-2-methylpropionate-pyruvate transaminase mitochondrion - 5739 737947 2.6.1.41 D-methionine-pyruvate transaminase mitochondrion - 5739 636704 2.6.1.42 branched-chain-amino-acid transaminase mitochondrion - 5739 -, 639993, 639996, 639997, 640014, 640017, 640019, 640021, 640022, 640024, 640025, 640029, 640031, 640034, 640035, 640037, 640038, 640039, 640040, 640042, 640043, 662423, 674728, 675563, 705527, 721137, 723026, 723462, 738842, 739398, 758607, 758657, 758690, 758728, 759414, 759594, 759639, 759717, 759733, 759947 2.6.1.42 branched-chain-amino-acid transaminase mitochondrion inner membrane-matrix 5739 639998 2.6.1.42 branched-chain-amino-acid transaminase mitochondrion isoform Atbcat-1 5739 663104 2.6.1.42 branched-chain-amino-acid transaminase mitochondrion mainly located in the cytoplasm 5739 639996 2.6.1.42 branched-chain-amino-acid transaminase mitochondrion mitochondrial matrix 5739 640022, 640026 2.6.1.43 aminolevulinate transaminase mitochondrion - 5739 -, 640046, 640047, 640051, 640053, 640055, 640057, 640061 2.6.1.43 aminolevulinate transaminase mitochondrion different isozymes in cytosol and mitochondrion 5739 -, 640060 2.6.1.43 aminolevulinate transaminase mitochondrion mitochondrial matrix 5739 -, 640058, 640062 2.6.1.44 alanine-glyoxylate transaminase mitochondrion - 5739 -, 640065, 640067, 640073, 640080, 640082, 704697, 721464, 759097 2.6.1.44 alanine-glyoxylate transaminase mitochondrion apoenzyme in the peroxisomes, holoenzyme in the mitochondrion 5739 640074 2.6.1.44 alanine-glyoxylate transaminase mitochondrion mutant enzyme G170R 5739 676835 2.6.1.44 alanine-glyoxylate transaminase mitochondrion the first 93 nucleotides of the full-length cDNA are predicted to encode the signal sequence that denote localization to the mitochondria 5739 -, 759951 2.6.1.5 tyrosine transaminase mitochondrion - 5739 640098 2.6.1.51 serine-pyruvate transaminase mitochondrion - 5739 -, 640077, 640082, 640135, 640136, 640138, 640139, 640141, 640142, 640143, 640144, 640146, 640148, 640149 2.6.1.51 serine-pyruvate transaminase mitochondrion induction by glucagon, leading to reduced formation of oxalate from L-hydroxyproline 5739 658995 2.6.1.58 phenylalanine(histidine) transaminase mitochondrion mitochondrial fraction: 70% of enzymatic activity, cytosolic fraction: 12% 5739 636975 2.6.1.6 leucine transaminase mitochondrion - 5739 639996, 640014, 640166 2.6.1.60 aromatic-amino-acid-glyoxylate transaminase mitochondrion - 5739 -, 636995, 637008 2.6.1.60 aromatic-amino-acid-glyoxylate transaminase mitochondrion matrix 5739 637008 2.6.1.62 adenosylmethionine-8-amino-7-oxononanoate transaminase mitochondrion - 5739 723412 2.6.1.62 adenosylmethionine-8-amino-7-oxononanoate transaminase mitochondrion mitochondrial matrix 5739 723412 2.6.1.63 kynurenine-glyoxylate transaminase mitochondrion - 5739 -, 636995, 636996 2.6.1.64 glutamine-phenylpyruvate transaminase mitochondrion - 5739 -, 288890, 636626, 636642, 640174, 640175, 640177, 640179 2.6.1.7 kynurenine-oxoglutarate transaminase mitochondrion - 5739 -, 439375, 636608, 636610, 636611, 636612, 636620, 636623, 721881, 738423, 759156 2.6.1.7 kynurenine-oxoglutarate transaminase mitochondrion 40% of kidney total activity 5739 636609 2.6.1.7 kynurenine-oxoglutarate transaminase mitochondrion 80% of liver total activity 5739 636609 2.6.1.7 kynurenine-oxoglutarate transaminase mitochondrion cDNA encodes for a kynurenine aminotransferase that is identical to a soluble form but carries an additional stretch of 32 amino acids resembling a mitochondrial import sequence 5739 636626 2.6.1.7 kynurenine-oxoglutarate transaminase mitochondrion deduced amino acid sequence contains a mitochondrial leader sequence 5739 636638 2.6.1.7 kynurenine-oxoglutarate transaminase mitochondrion KAT IV 5739 703133 2.6.1.96 4-aminobutyrate-pyruvate transaminase mitochondrion - 5739 -, 637079, 704874, 704882, 720325, 723611, 739265 2.6.1.96 4-aminobutyrate-pyruvate transaminase mitochondrion GABA:pyruvate-T contains a mitochondrial signal sequence 5739 719204 2.6.1.96 4-aminobutyrate-pyruvate transaminase mitochondrion predicted 5739 738113 2.7.1.1 hexokinase mitochondrion - 5739 640243, 676731, 701997, 721777, 739283, 758656, 759445 2.7.1.1 hexokinase mitochondrion 15% of total placenta hexokinase activity, only hexokinase I 5739 640237 2.7.1.1 hexokinase mitochondrion association of hexokinase II with mitochondria can be inhibited by methyl jasmonate or with clotrimazole 5739 758656 2.7.1.1 hexokinase mitochondrion bound at type A sites and type B sites of brain mitochondria, ratio of type A:type B is 40:60, enzyme bound at type A sites is releaved by glucose 6-phosphate, but not that of type B sites 5739 640267 2.7.1.1 hexokinase mitochondrion glucokinase shows a mitochondrial localization upon removal of glucose 5739 702119 2.7.1.1 hexokinase mitochondrion hexokinase 2 is overexpressed in all aggressive tumors and predominantly found on the outer mitochondrial membrane. Structural and biochemical characterization of the mitochondrial conformation reveals higher conformational stability and slow protein unfolding rate compared with the cytosolic conformation. The interaction of hexokinase 2 with the mitochondria through its N-half is proposed to facilitate higher stability on the mitochondria 5739 758942 2.7.1.1 hexokinase mitochondrion hexokinase is functionally coupled to mitochondrial respiration 5739 759555 2.7.1.1 hexokinase mitochondrion hexokinase type I 5739 704456 2.7.1.1 hexokinase mitochondrion hexokinases A and B 5739 640233 2.7.1.1 hexokinase mitochondrion HKI remains strongly bound to mitochondria 5739 723530 2.7.1.1 hexokinase mitochondrion HKII translocates between mitochondria and the cytosol in response to glucose, glucose 6-phosphate and Akt 5739 723530 2.7.1.1 hexokinase mitochondrion key role for hexokinase activity and/or localization to the mitochondria in the regulation of neurite outgrowth in cultured adult sensory neurons 5739 689221 2.7.1.1 hexokinase mitochondrion oleate decreases hexokinase-mitochondrial binding and abolishes insulin-mediated translocation of HK I. Under basal conditions, the fraction of hexokinase I that is mitochondrially bound is 5times greater than for hexokinase II. Insulin and ischemia cause a fourfold increase in hexokinase II binding but only a doubling of hexokinase I binding 5739 671249 2.7.1.1 hexokinase mitochondrion the majority of the green fluorescent protein tagged HxkC appears to be associated with mitochondria both in cells grown in medium containing glucose as a carbon source and cells subjected to carbon starvation 5739 689000 2.7.1.1 hexokinase mitochondrion the mitochondrial membrane exopolyphosphatase activity is stimulated by the hexokinase reaction product, glucose-6-phosphate. Polyphosphates increase hydrogen peroxide generation in mitochondria in a situation where mitochondrial hexokinase is also active 5739 738409 2.7.1.1 hexokinase mitochondrion type I isozyme, binds with a hydrophobic N-terminal sequence to mitochondria through interaction with porin using intermitochondrial ATP as cosubstrate facilitating the coordination of the introduction of glucose into glycolysis, type II isozyme binds with a hydrophobic N-terminal sequence to mitochondria in vitro, in vivo preferably in cancer cells 5739 662531 2.7.1.1 hexokinase mitochondrion under basal conditions, the fraction of hexokinase I that is mitochondrially bound is 5times greater than for hexokinase II. Insulin and ischemia cause a fourfold increase in hexokinase II binding but only a doubling of hexokinase I binding 5739 671249 2.7.1.108 dolichol kinase mitochondrion - 5739 -, 640393 2.7.1.113 deoxyguanosine kinase mitochondrion - 5739 640550, 640554, 640555, 640556, 640558, 640559, 661040, 661728, 661731, 662882, 662992, 671801, 671890, 673472, 703966, 705750, 737461, 737599, 738348, 760985, 762058 2.7.1.113 deoxyguanosine kinase mitochondrion deoxyguanosine kinase is encoded by the nuclear DNA and transported into the mitochondria 5739 723198 2.7.1.113 deoxyguanosine kinase mitochondrion enzyme possesses an N-terminal mitochondrial import signal 5739 661453 2.7.1.113 deoxyguanosine kinase mitochondrion membrane associated 5739 640551 2.7.1.113 deoxyguanosine kinase mitochondrion mitochondrial deoxguanosine kinase is redistributed to the cytosol during apoptosis 5739 640560 2.7.1.113 deoxyguanosine kinase mitochondrion N-terminal enzyme sequence contains a mitochondrial import signal 5739 663023 2.7.1.138 ceramide kinase mitochondrion - 5739 662592, 675274, 691938 2.7.1.138 ceramide kinase mitochondrion in testis 5739 662592 2.7.1.145 deoxynucleoside kinase mitochondrion - 5739 676100 2.7.1.145 deoxynucleoside kinase mitochondrion Dm-dNK localizes to the nucleus through a mitochondrial localization signal 5739 739211 2.7.1.147 ADP-specific glucose/glucosamine kinase mitochondrion - 5739 721992 2.7.1.160 2'-phosphotransferase mitochondrion 2'-phosphotransferase shows a tendency to associate with mitochondria 5739 685412 2.7.1.21 thymidine kinase mitochondrion - 5739 641125, 641126, 641133, 641138, 641139, 641142, 641143, 641150, 661066, 722909, 723302, 723324 2.7.1.21 thymidine kinase mitochondrion beta-thymidine kinase-2 5739 705803 2.7.1.21 thymidine kinase mitochondrion thymidine kinase 2 5739 723301 2.7.1.23 NAD+ kinase mitochondrion - 5739 691390, 723257, 759283 2.7.1.23 NAD+ kinase mitochondrion the Ca2+-calmodulin-dependent NAD+ kinase isoforms, amongst which is the isoform bound to mitochondrial membranes play an important role at the end of sensu stricto germination and during the following growth of Avena sativa 5739 641198 2.7.1.23 NAD+ kinase mitochondrion the enzyme from skeletal muscle is susceptible to seasonal alterations: in autumn and winter, NADP+ synthesis proceeds maximally in the cytoplasm, in spring, higher activity is found in the mitochondrial fraction 5739 641202 2.7.1.24 dephospho-CoA kinase mitochondrion - 5739 643174, 645067 2.7.1.24 dephospho-CoA kinase mitochondrion inner membrane 5739 643179 2.7.1.24 dephospho-CoA kinase mitochondrion intramitochondrial localization 5739 643180 2.7.1.26 riboflavin kinase mitochondrion - 5739 703642 2.7.1.30 glycerol kinase mitochondrion - 5739 641287, 641291, 761751 2.7.1.30 glycerol kinase mitochondrion isoform GK+EX18 5739 660906 2.7.1.31 glycerate 3-kinase mitochondrion - 5739 673281 2.7.1.32 choline kinase mitochondrion - 5739 641362 2.7.1.33 pantothenate kinase mitochondrion - 5739 671347, 676884, 703958, 759477 2.7.1.33 pantothenate kinase mitochondrion one of the splicing isoforms localizes to mitochondria 5739 703958 2.7.1.33 pantothenate kinase mitochondrion PANK2 contains a mitochondrial targeting signal 5739 660712 2.7.1.33 pantothenate kinase mitochondrion PanK2 is located in the mitochondria to sense the levels of palmitoylcarnitine and up-regulate CoA biosynthesis in response to an increased mitochondrial demand for the cofactor to support beta-oxidation 5739 676884 2.7.1.33 pantothenate kinase mitochondrion several isoforms of brain PanK2 5739 662687 2.7.1.40 pyruvate kinase mitochondrion - 5739 708992, 710195 2.7.1.40 pyruvate kinase mitochondrion mitochondrial phosphoenolpyruvate kinase is restricted to the matrix 5739 707282 2.7.1.59 N-acetylglucosamine kinase mitochondrion - 5739 723600 2.7.1.73 inosine kinase mitochondrion intermembrane space 5739 641923 2.7.1.74 deoxycytidine kinase mitochondrion - 5739 641945, 641949, 762058 2.7.1.74 deoxycytidine kinase mitochondrion not 5739 641943 2.7.1.74 deoxycytidine kinase mitochondrion outer membrane 5739 641938 2.7.1.76 2'-deoxyadenosine kinase mitochondrion - 5739 739204 2.7.1.77 nucleoside phosphotransferase mitochondrion - 5739 641138 2.7.1.86 NADH kinase mitochondrion - 5739 -, 642062, 642063, 649041, 674616, 691390, 695829, 705593, 721139, 721392, 722693, 722719 2.7.1.86 NADH kinase mitochondrion enzyme functions exclusively in mitochondria 5739 649040 2.7.1.86 NADH kinase mitochondrion localized exclusively in mitochondria 5739 -, 642066 2.7.1.86 NADH kinase mitochondrion the enzyme is localized and functions at the intermembrane space side of the inner membrane 5739 -, 642064 2.7.1.94 acylglycerol kinase mitochondrion - 5739 662505, 674834, 723199, 737779, 739046, 739084, 761886, 762499 2.7.10.2 non-specific protein-tyrosine kinase mitochondrion - 5739 738284 2.7.11.1 non-specific serine/threonine protein kinase mitochondrion - 5739 645086 2.7.11.1 non-specific serine/threonine protein kinase mitochondrion two mitochondrial kinases I and II and a cytosolic kinase 5739 645089 2.7.11.11 cAMP-dependent protein kinase mitochondrion - 5739 -, 674804, 674837, 721663, 721701 2.7.11.11 cAMP-dependent protein kinase mitochondrion associated with the inner membrane/matrix 5739 721744 2.7.11.11 cAMP-dependent protein kinase mitochondrion localization of cAMP-dependent protein kinase, composed of the components C-PKA, R-PKA, and AKAP121, associated with the A-kinase anchor protein in the inner mitochondrial compartment 5739 673668 2.7.11.15 beta-adrenergic-receptor kinase mitochondrion after myocardial ischemic and oxidative stress there is increased mitochondrial GRK2 translocation 5739 724779 2.7.11.2 [pyruvate dehydrogenase (acetyl-transferring)] kinase mitochondrion - 5739 -, 348935, 642133, 642138, 642144, 642146, 642155, 642156, 642161, 642162, 642164, 660923, 660995, 661094, 661219, 661961, 661963, 662397, 662537, 672273, 672978, 673007, 673162, 673647, 673688, 674640, 674730, 677167, 705630, 722780, 722788, 723399, 738178, 739451, 739698, 760260, 761193 2.7.11.2 [pyruvate dehydrogenase (acetyl-transferring)] kinase mitochondrion enzyme contains a 18-amino acid mitochondrial import signal sequence 5739 642151 2.7.11.2 [pyruvate dehydrogenase (acetyl-transferring)] kinase mitochondrion inner membrane matrix compartment 5739 642137, 94884 2.7.11.2 [pyruvate dehydrogenase (acetyl-transferring)] kinase mitochondrion protein x serves to anchor the kinase to the core of the complex 5739 642141 2.7.11.2 [pyruvate dehydrogenase (acetyl-transferring)] kinase mitochondrion purified pyruvate dehydrogenase-complex contains 3 molecules of kinase, but one molecule of dihydrolipoyl transacetylase-protein x-subcomplex of pyruvate dehydrogenase activates more than 15 molecules of kinase 5739 642141 2.7.11.2 [pyruvate dehydrogenase (acetyl-transferring)] kinase mitochondrion tightly bound to dihydrolipoamide acetyltransferase of pyruvate dehydrogenase complex 5739 642136, 642137, 94884, 94893 2.7.11.22 cyclin-dependent kinase mitochondrion - 5739 666124, 691679 2.7.11.24 mitogen-activated protein kinase mitochondrion - 5739 740495 2.7.11.24 mitogen-activated protein kinase mitochondrion p38 MAPK 5739 666333 2.7.11.25 mitogen-activated protein kinase kinase kinase mitochondrion - 5739 760789 2.7.11.26 tau-protein kinase mitochondrion GSK-3beta partially localizes within mitochondria 5739 706424 2.7.11.31 [hydroxymethylglutaryl-CoA reductase (NADPH)] kinase mitochondrion - 5739 691556 2.7.11.4 [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase mitochondrion - 5739 -, 349051, 587061, 640575, 640576, 640583, 640587, 640588, 640590, 640591, 640596, 640599, 660683, 662283, 739455, 739536, 760495, 761232, 94884 2.7.11.4 [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase mitochondrion part of intramitochondrial branched-chain 2-oxoacid dehydrogenase complex 5739 349056 2.7.11.8 Fas-activated serine/threonine kinase mitochondrion - 5739 761639 2.7.11.8 Fas-activated serine/threonine kinase mitochondrion enzyme has a mitochondrial targeting domain 5739 660872, 662849 2.7.11.8 Fas-activated serine/threonine kinase mitochondrion localizes to mitochondrial RNA granules 5739 738004 2.7.12.2 mitogen-activated protein kinase kinase mitochondrion - 5739 741160 2.7.2.2 carbamate kinase mitochondrion - 5739 642216 2.7.2.3 phosphoglycerate kinase mitochondrion - 5739 762240 2.7.2.8 acetylglutamate kinase mitochondrion - 5739 -, 642357, 761811 2.7.3.2 creatine kinase mitochondrion - 5739 -, 642372, 642373, 642374, 642376, 642377, 642383, 642385, 642388, 642393, 642395, 642401, 642403, 642410, 642417, 642422, 642430, 642440, 661220, 661271, 661528, 662511, 671632, 672391, 674039, 703836, 705501, 706831, 722684, 737427, 739050, 760407, 761841 2.7.3.2 creatine kinase mitochondrion accumulated in contact sites between inner and outer mitochondrial membrane 5739 642371, 642375 2.7.3.2 creatine kinase mitochondrion association with by brain-type creatine kinase 5739 737427 2.7.3.2 creatine kinase mitochondrion HaCaT cells express low levels of cytoplasmic creatine kinase (BB-CK) and high levels of mitochondrial creatine kinase (uMiCK). HeLa-S3 cells express high levels of cytoplasmic creatine kinase (BB-CK) and low levels of mitochondrial creatine kinase (uMiCK). The mitochondrial CK isoform plays the most crucial role in maintaining cell viability by stabilizing contact sites between inner and outer mitochondrial membranes and maintaining local metabolite channeling, thus avoiding transition pore opening which eventually results in activation of caspase cell-death pathways 5739 675957 2.7.3.2 creatine kinase mitochondrion head isozyme 5739 642418 2.7.3.2 creatine kinase mitochondrion in mitochondria from intact animals, mCK exists as a mixture of two oligomeric forms (dimer and octamer: 68 and 32%, respectively) 5739 721956 2.7.3.2 creatine kinase mitochondrion mitochondrial isoform plays the most crucial role in maintaining cell viability by stabilizing contact sites between inner and outer mitochondrial membranse and maintaining local metabolic channeling 5739 675957 2.7.3.2 creatine kinase mitochondrion mitochondrial isozyme MtCK 5739 661523 2.7.3.2 creatine kinase mitochondrion octameric MtCK is situated in the mitochondrial intermembrane space, binding simultaneously to both mitochondrial inner and outer membranes, as well as in the cristae space bound to inner membrane 5739 737427 2.7.3.2 creatine kinase mitochondrion overview on intramitochondrial localization 5739 642438 2.7.3.2 creatine kinase mitochondrion respiration rate at 10-20°C, pH 7.1-7.3 5739 662524 2.7.3.2 creatine kinase mitochondrion sarcomeric, mitochondrial isoform sMiCK 5739 689889 2.7.3.2 creatine kinase mitochondrion simulation of hyperglycemic conditions induces H2O2 production in a creatine sensitive manner 5739 674713 2.7.3.2 creatine kinase mitochondrion there are two mitochondrial isoforms of CK: s-type (sarcomeric, expressed in skeletal and heart muscles only) and u-type (ubiquitous, expressed in many other tissues), which both exist as octamers in fish as well as in higher vertebrates and invertebrates. The mitochondrial s-type, rather than u-type, is predominantly expressed in herring eye 5739 738079 2.7.3.3 arginine kinase mitochondrion no microcompartmentation 5739 642479 2.7.3.3 arginine kinase mitochondrion TcAK1 is detected in cytoplasm including mitochondria 5739 760906 2.7.3.4 taurocyamine kinase mitochondrion - 5739 642485, 661697, 661899, 675484, 721715 2.7.3.4 taurocyamine kinase mitochondrion annelid-specific enzyme 5739 691916 2.7.3.5 lombricine kinase mitochondrion - 5739 675484 2.7.4.10 nucleoside-triphosphate-adenylate kinase mitochondrion - 5739 721237, 722281, 737551 2.7.4.10 nucleoside-triphosphate-adenylate kinase mitochondrion matrix 5739 642534, 642535, 642536, 642537, 762326 2.7.4.14 UMP/CMP kinase mitochondrion - 5739 761290 2.7.4.14 UMP/CMP kinase mitochondrion N-terminal signal targets the enzyme to mitochondria 5739 645155 2.7.4.14 UMP/CMP kinase mitochondrion UMP-CMPK2 contains an N-terminal mitochondrial targeting sequence 5739 693063 2.7.4.15 thiamine-diphosphate kinase mitochondrion primarily localized in 5739 645164 2.7.4.21 inositol-hexakisphosphate 5-kinase mitochondrion - 5739 662355 2.7.4.3 adenylate kinase mitochondrion - 5739 642559, 642560, 642600, 642602, 673317, 704047, 705850, 721943, 722732, 761162, 761742 2.7.4.3 adenylate kinase mitochondrion after 2 h from heat shock, rate of ADT/ATP exchange by adenine nucleotide translocator activity, adenylate kinase activity and nucleoside diphosphate kinase EC 2.7.4.6 are inhibitied in a non-competitive-like manner. Externally added ascorbate prevents the inhibition 5739 672407 2.7.4.3 adenylate kinase mitochondrion cytosolic and mitochondrial kinases are distinct isozymes 5739 642568, 642572 2.7.4.3 adenylate kinase mitochondrion intermembrane space 5739 642558, 642568, 642620, 762326 2.7.4.3 adenylate kinase mitochondrion isofrms AMK21and AMK7 5739 694715 2.7.4.3 adenylate kinase mitochondrion liver enzyme 5739 642595 2.7.4.3 adenylate kinase mitochondrion localized in the mitochondrial intermembrane space 5739 703297 2.7.4.3 adenylate kinase mitochondrion most highly expressed isoform AK1 5739 694756 2.7.4.4 nucleoside-phosphate kinase mitochondrion - 5739 642628 2.7.4.6 nucleoside-diphosphate kinase mitochondrion - 5739 642661, 642683, 663130, 694579, 694756, 701452, 721737, 761777 2.7.4.6 nucleoside-diphosphate kinase mitochondrion after 2 h from heat shock, rate of ADT/ATP exchange by adenine nucleotide translocator activity, adenylate kinase activity EC 2.7.4.3 and nucleoside diphosphate kinase are inhibited in a non-competitive-like manner. Externally added ascorbate prevents the inhibition 5739 672407 2.7.4.6 nucleoside-diphosphate kinase mitochondrion dual cellular localization in mitochondria and cytosol 5739 642637 2.7.4.6 nucleoside-diphosphate kinase mitochondrion intermembrane space 5739 673695 2.7.4.6 nucleoside-diphosphate kinase mitochondrion intermembrane space of mitochondria 5739 673695 2.7.4.6 nucleoside-diphosphate kinase mitochondrion isozyme NDPK3 5739 721870 2.7.4.6 nucleoside-diphosphate kinase mitochondrion isozyme NDPK3a is predominantly localized in the mictochondria 5739 694661 2.7.4.6 nucleoside-diphosphate kinase mitochondrion located in the mitochondrial intermembrane/cristae space 5739 702417 2.7.4.6 nucleoside-diphosphate kinase mitochondrion matrix space 5739 642671 2.7.4.6 nucleoside-diphosphate kinase mitochondrion slightly 5739 692239 2.7.4.8 guanylate kinase mitochondrion - 5739 642694 2.7.4.8 guanylate kinase mitochondrion plastid/mitochondrial isozyme 5739 700744 2.7.4.9 dTMP kinase mitochondrion - 5739 705442, 760006 2.7.4.9 dTMP kinase mitochondrion activity detected in the outer membrane fraction may originate from a novel dTMP kinase, distinct from the canonical TYMK 5739 759870 2.7.4.9 dTMP kinase mitochondrion isoform TMPK2, mitochondrial import sequence at its N-terminus and mitochondrial localization of Green Fluorescent Protein fusion protein 5739 692491 2.7.4.9 dTMP kinase mitochondrion longer isoform 5739 694670 2.7.4.9 dTMP kinase mitochondrion no activity in mitochondria 5739 645191 2.7.4.9 dTMP kinase mitochondrion the enzyme is loosely bound to mitochondrial membrane 5739 645195 2.7.6.1 ribose-phosphate diphosphokinase mitochondrion - 5739 642736 2.7.6.1 ribose-phosphate diphosphokinase mitochondrion 80% of the liver enzyme is found in the mitochondria 5739 642710 2.7.6.3 2-amino-4-hydroxy-6-hydroxymethyldihydropteridine diphosphokinase mitochondrion - 5739 674784 2.7.6.3 2-amino-4-hydroxy-6-hydroxymethyldihydropteridine diphosphokinase mitochondrion mitHPPK/DHPS 5739 674784 2.7.7.1 nicotinamide-nucleotide adenylyltransferase mitochondrion - 5739 642805, 703814, 705288 2.7.7.1 nicotinamide-nucleotide adenylyltransferase mitochondrion isoform NMAT3 5739 722676 2.7.7.1 nicotinamide-nucleotide adenylyltransferase mitochondrion isozyme NMNAT3 5739 672248 2.7.7.1 nicotinamide-nucleotide adenylyltransferase mitochondrion NMNAT3 5739 662419, 672248, 675543 2.7.7.1 nicotinamide-nucleotide adenylyltransferase mitochondrion PNAT-3 5739 642795 2.7.7.102 DNA primase AEP mitochondrion - 5739 751551 2.7.7.108 protein adenylyltransferase mitochondrion - 5739 764427 2.7.7.13 mannose-1-phosphate guanylyltransferase mitochondrion expression of gene OsMPG2 5739 723440 2.7.7.14 ethanolamine-phosphate cytidylyltransferase mitochondrion - 5739 660979, 676425 2.7.7.18 nicotinate-nucleotide adenylyltransferase mitochondrion - 5739 703814 2.7.7.18 nicotinate-nucleotide adenylyltransferase mitochondrion isoform NMNAT3 is targeted to mitochondria by its N-terminus 5739 722676 2.7.7.19 polynucleotide adenylyltransferase mitochondrion - 5739 642951, 642965, 642967, 662366, 662974, 691063, 739199, 739200, 760987 2.7.7.2 FAD synthase mitochondrion - 5739 -, 705590, 738594 2.7.7.2 FAD synthase mitochondrion membrane-enriched fraction 5739 703642 2.7.7.2 FAD synthase mitochondrion presence of mitochondrial FAD synthetase activity in strains transformed with FAD1 on a high-copy-number plasmid, but not in mitochondria of wild-type strains 5739 643001 2.7.7.3 pantetheine-phosphate adenylyltransferase mitochondrion - 5739 643174, 643175 2.7.7.3 pantetheine-phosphate adenylyltransferase mitochondrion inner membrane 5739 643179 2.7.7.3 pantetheine-phosphate adenylyltransferase mitochondrion intramitochondrial localization 5739 643180 2.7.7.38 3-deoxy-manno-octulosonate cytidylyltransferase mitochondrion - 5739 723388 2.7.7.4 sulfate adenylyltransferase mitochondrion - 5739 738302 2.7.7.41 phosphatidate cytidylyltransferase mitochondrion - 5739 -, 760639, 761129, 762488 2.7.7.41 phosphatidate cytidylyltransferase mitochondrion 5-10% of the cellular activity 5739 643319 2.7.7.41 phosphatidate cytidylyltransferase mitochondrion enzyme is associated with both, the mitochondria and the endoplasmic reticulum 5739 643321 2.7.7.41 phosphatidate cytidylyltransferase mitochondrion inner mitochondrial membrane 5739 643302 2.7.7.41 phosphatidate cytidylyltransferase mitochondrion isoform Tam41 directly catalyzes the formation of CDP-diacylglycerol from phosphatidic acid in the mitochondrial inner membrane 5739 738000 2.7.7.41 phosphatidate cytidylyltransferase mitochondrion main site of localization 5739 643301 2.7.7.45 guanosine-triphosphate guanylyltransferase mitochondrion small amount of activity 5739 643338 2.7.7.49 RNA-directed DNA polymerase mitochondrion - 5739 643430, 691140, 692794 2.7.7.52 RNA uridylyltransferase mitochondrion - 5739 -, 1997, 643501, 643502, 643503, 675809, 722964, 722966 2.7.7.52 RNA uridylyltransferase mitochondrion 2 isozymes RET1 and RET2 in mitochondria editosomes 5739 643506 2.7.7.52 RNA uridylyltransferase mitochondrion RET1 5739 691064 2.7.7.52 RNA uridylyltransferase mitochondrion TUT1 and TUT2 5739 691064 2.7.7.56 tRNA nucleotidyltransferase mitochondrion partial localisation of full-length protein and isoform with translational start at ATG2 (Met6), targeting dependent not only on N-terminal 68 amino acids 5739 693409 2.7.7.6 DNA-directed RNA polymerase mitochondrion - 5739 673196, 703150, 703854, 721721, 723183, 738671, 738689, 760357 2.7.7.6 DNA-directed RNA polymerase mitochondrion N-terminal transit peptide of SmRpoT confers enzyme targeting to mitochondria 5739 705208 2.7.7.6 DNA-directed RNA polymerase mitochondrion the enzyme in nucleus encoded and then transported to the mitochondrion 5739 705687 2.7.7.7 DNA-directed DNA polymerase mitochondrion - 5739 -, 643424, 643625, 643642, 643645, 643647, 643649, 662379, 671388, 690469, 705778, 705867, 706062, 721707, 723108, 723249 2.7.7.7 DNA-directed DNA polymerase mitochondrion in yeast mitochondria two different DNA polymerases exist, pol gamma and pol NI, i.e. DNA polymerase alpha catalytic subunit A and its associated DNA polymerase subunit alphabeta, POL12 5739 -, 721767 2.7.7.72 CCA tRNA nucleotidyltransferase mitochondrion - 5739 738350, 739230, 739631 2.7.7.72 CCA tRNA nucleotidyltransferase mitochondrion a fraction of CAE is imported into mitochondria 5739 -, 761522 2.7.7.72 CCA tRNA nucleotidyltransferase mitochondrion both isoforms Cca1 and CCa2 are not specifically enriched in mitochondria 5739 761303 2.7.7.72 CCA tRNA nucleotidyltransferase mitochondrion human mitochondrial tRNA-nucleotidyltransferase 5739 643052 2.7.7.72 CCA tRNA nucleotidyltransferase mitochondrion the first five amino acids of the N-terminal signal sequence are required for efficient translocation into mitochondria. Variants lacking the first start codon show less mitochondrial import. Mutations in the mature domain reduce the localization tomitochondria and in turn enhance the translocation into chloroplasts 5739 737637 2.7.7.79 tRNAHis guanylyltransferase mitochondrion - 5739 713148, 760493, 761202 2.7.7.8 polyribonucleotide nucleotidyltransferase mitochondrion - 5739 643680, 643693, 643695, 662366, 662972, 690735, 709034, 710283, 723628, 761251, 761702 2.7.7.8 polyribonucleotide nucleotidyltransferase mitochondrion basal and interferon-beta induced enzyme is efficiently imported into mitochondria with coupled processing of the N-terminal targeting sequence. It localizes to the intermembrane space as a peripheral membrane protein in a multimeric complex 5739 675974 2.7.7.8 polyribonucleotide nucleotidyltransferase mitochondrion located to intermembrane space 5739 695110 2.7.7.8 polyribonucleotide nucleotidyltransferase mitochondrion mammalian PNPase contains an N-terminal mitochondrial localization signal facilitating its subcellular localization in mitochondria 5739 723310 2.7.7.84 2'-5' oligoadenylate synthase mitochondrion OAS1 p46 isoform 5739 762506 2.7.8.10 sphingosine cholinephosphotransferase mitochondrion - 5739 643758 2.7.8.11 CDP-diacylglycerol-inositol 3-phosphatidyltransferase mitochondrion - 5739 645302, 645308 2.7.8.11 CDP-diacylglycerol-inositol 3-phosphatidyltransferase mitochondrion sub-cellular localization of PIS is studied in Solanum tuberosum by membrane fractionation, enzymatic analysis and by confocal microscopy in living cells. In potato, beside the endoplasmic reticulum, the activity of PIS is found to be tightly associated to mitochondria, more specifically to the outer mitochondrial membrane 5739 726195 2.7.8.2 diacylglycerol cholinephosphotransferase mitochondrion - 5739 644329, 644333, 662076 2.7.8.2 diacylglycerol cholinephosphotransferase mitochondrion located on outside of membrane 5739 644325 2.7.8.29 L-serine-phosphatidylethanolamine phosphatidyltransferase mitochondrion PSS2 of CHO-K1 cells is present only in mitochondria-associated membranes 5739 717762 2.7.8.29 L-serine-phosphatidylethanolamine phosphatidyltransferase mitochondrion PSS2 of CHO-K1 cells is restricted to mitochondria-associated membranes 5739 717762 2.7.8.3 ceramide cholinephosphotransferase mitochondrion - 5739 645384, 645387 2.7.8.41 cardiolipin synthase (CMP-forming) mitochondrion - 5739 -, 727054, 727056, 727507, 728087, 728227, 739118, 760627, 761083, 761829 2.7.8.41 cardiolipin synthase (CMP-forming) mitochondrion mitochondrial membrane 5739 727808 2.7.8.41 cardiolipin synthase (CMP-forming) mitochondrion the recombinant enzyme is exclusively localized in mitochondria when expressed in COS-7 cells 5739 726900 2.7.8.5 CDP-diacylglycerol-glycerol-3-phosphate 1-phosphatidyltransferase mitochondrion - 5739 644288, 644298, 644299, 661805, 692419, 694080, 703786, 739659, 94808 2.7.8.5 CDP-diacylglycerol-glycerol-3-phosphate 1-phosphatidyltransferase mitochondrion CLS1 sequence contains a putative N-terminal mitochondrial targeting sequence 5739 761150 2.7.8.5 CDP-diacylglycerol-glycerol-3-phosphate 1-phosphatidyltransferase mitochondrion crude mitochondrial fraction   5739 644287 2.7.8.5 CDP-diacylglycerol-glycerol-3-phosphate 1-phosphatidyltransferase mitochondrion inner membrane 5739 644300, 644301 2.7.8.5 CDP-diacylglycerol-glycerol-3-phosphate 1-phosphatidyltransferase mitochondrion membrane 5739 644293 2.7.8.5 CDP-diacylglycerol-glycerol-3-phosphate 1-phosphatidyltransferase mitochondrion mitochondrial inner membrane 5739 -, 723221 2.7.8.5 CDP-diacylglycerol-glycerol-3-phosphate 1-phosphatidyltransferase mitochondrion PGP1 5739 738231 2.7.8.7 holo-[acyl-carrier-protein] synthase mitochondrion - 5739 -, 722318, 723227, 723231, 723538, 723567, 723610 2.7.8.7 holo-[acyl-carrier-protein] synthase mitochondrion isoenzyme, mutational loss of mitochondrial ACP has no effect on bulk cellular fatty acid synthesis 5739 645405 2.7.8.7 holo-[acyl-carrier-protein] synthase mitochondrion the mitochondrial localization of the AT3G11470-encoded proteins is validated by the ability of their N-terminal 80-residue leader sequence to guide a chimeric GFP protein to this organelle 5739 739319 2.8.1.1 thiosulfate sulfurtransferase mitochondrion - 5739 645498, 645502, 645538, 645539, 645540, 645546, 725406, 738551, 739155, 762111 2.8.1.1 thiosulfate sulfurtransferase mitochondrion mammalian liver enzyme occurs exclusively in mitochondrial matrix 5739 645503 2.8.1.1 thiosulfate sulfurtransferase mitochondrion two isoforms: a negative and a less negative form of rhodanese 5739 645524 2.8.1.2 3-mercaptopyruvate sulfurtransferase mitochondrion - 5739 645498, 645551, 645557, 645561, 645562, 645565, 723958, 724194, 724231, 738551, 738896, 738988, 739651, 760675, 762111 2.8.1.2 3-mercaptopyruvate sulfurtransferase mitochondrion highest activity in the matrix, followed by intramembrane space, low activity in inner and outer membrane 5739 -, 645554 2.8.1.2 3-mercaptopyruvate sulfurtransferase mitochondrion isoforms TUM1-Iso2 5739 738634 2.8.1.2 3-mercaptopyruvate sulfurtransferase mitochondrion partly 5739 760854 2.8.1.2 3-mercaptopyruvate sulfurtransferase mitochondrion two splice variants of MPST, differing by 20 amino acids at the N-terminus, give rise to the cytosolic MPST1 and mitochondrial MPST2 isoforms 5739 761523 2.8.1.6 biotin synthase mitochondrion - 5739 645611, 738906 2.8.1.7 cysteine desulfurase mitochondrion - 5739 645625, 662190, 662693, 675971, 703682, 724238, 738144, 738601, 760587 2.8.1.7 cysteine desulfurase mitochondrion enzyme has an N-terminal targeting signal that directs the enzyme to mitochondria when expressed in Saccharomyces cerevisiae 5739 645639 2.8.1.7 cysteine desulfurase mitochondrion isoform Nfs1 5739 726016 2.8.1.7 cysteine desulfurase mitochondrion mitochondrial Nfs1p is required for the biogenesis of both mitochondrial and extramitochondrial Fe/S proteins 5739 662257 2.8.1.7 cysteine desulfurase mitochondrion predominantly localized in the mitochondria 5739 726333 2.8.1.7 cysteine desulfurase mitochondrion predominat localization of isoform Nfs1 5739 675971 2.8.1.8 lipoyl synthase mitochondrion - 5739 662098, 762170 2.8.2.13 psychosine sulfotransferase mitochondrion - 5739 643818 2.8.2.19 triglucosylalkylacylglycerol sulfotransferase mitochondrion - 5739 643819, 643820 2.8.3.13 succinate-hydroxymethylglutarate CoA-transferase mitochondrion - 5739 643833, 643834, 738808 2.8.3.13 succinate-hydroxymethylglutarate CoA-transferase mitochondrion mainly in the mitochondrial matrix, also in the inner membrane 5739 643832 2.8.3.27 propanoyl-CoA:succinate CoA transferase mitochondrion - 5739 764526 2.8.3.27 propanoyl-CoA:succinate CoA transferase mitochondrion anaerobic mitochondria 5739 765369 2.8.3.5 3-oxoacid CoA-transferase mitochondrion - 5739 645971, 645973, 645976, 645977, 645978, 645988, 645993, 645994, 661256, 672216, 703384, 703810, 705523, 724109, 737420, 738807 2.8.3.5 3-oxoacid CoA-transferase mitochondrion predominantly 5739 645972 2.8.3.5 3-oxoacid CoA-transferase mitochondrion rat heart mitochondria at 4, 13, 19, and 24 months of age 5739 690880 2.8.3.5 3-oxoacid CoA-transferase mitochondrion the enzyme is a mitochondrial homodimer 5739 694193 2.8.3.6 3-oxoadipate CoA-transferase mitochondrion - 5739 -, 756853 2.8.3.8 acetate CoA-transferase mitochondrion - 5739 725472 3.1.1.1 carboxylesterase mitochondrion - 5739 651507 3.1.1.106 O-acetyl-ADP-ribose deacetylase mitochondrion - 5739 750645 3.1.1.118 phospholipid sn-1 acylhydrolase mitochondrion - 5739 761452 3.1.1.13 sterol esterase mitochondrion low activity 5739 651007 3.1.1.28 acylcarnitine hydrolase mitochondrion - 5739 80784, 80789 3.1.1.29 aminoacyl-tRNA hydrolase mitochondrion - 5739 730350 3.1.1.29 aminoacyl-tRNA hydrolase mitochondrion mitochondiral ribosome 5739 708331 3.1.1.32 phospholipase A1 mitochondrion - 5739 730037 3.1.1.32 phospholipase A1 mitochondrion mitochondrial matrix 5739 -, 749810 3.1.1.4 phospholipase A2 mitochondrion - 5739 133897, 133920, 709076, 750682 3.1.1.5 lysophospholipase mitochondrion enzyme is also located in mitochondria even in the absence of prenylation 5739 702426 3.1.1.67 fatty-acyl-ethyl-ester synthase mitochondrion enzyme with FAEES/carboxylesterase activity from heart 5739 646079 3.1.1.7 acetylcholinesterase mitochondrion - 5739 114196, 653108 3.1.1.7 acetylcholinesterase mitochondrion synaptosomal-mitochondrial fraction 5739 114136 3.1.1.8 cholinesterase mitochondrion - 5739 653108 3.1.13.1 exoribonuclease II mitochondrion - 5739 656028, 666793 3.1.13.3 oligonucleotidase mitochondrion dual cellular localization being present both in cytosolic and mitochondrial fractions. The mitochondrial form localizes to both the intermembrane space and the matrix 5739 730733 3.1.13.4 poly(A)-specific ribonuclease mitochondrion - 5739 -, 714876 3.1.13.4 poly(A)-specific ribonuclease mitochondrion mitochondrial localizations of isoform AHG2 and poly(A) polymerase AGS1 are required for their functions in the regulation of the poly(A) tract of mitochondrial mRNA 5739 730444 3.1.13.5 ribonuclease D mitochondrion predicted mitochondrial targeting sequence present at the N-terminus of TbRND 5739 -, 715578 3.1.14.1 yeast ribonuclease mitochondrion - 5739 651791, 652380, 666970 3.1.16.1 spleen exonuclease mitochondrion - 5739 134149 3.1.2.1 acetyl-CoA hydrolase mitochondrion - 5739 650340, 750619, 94468, 94469, 94470, 94471, 94478, 94479, 94481, 94487, 94488, 94490, 94495 3.1.2.1 acetyl-CoA hydrolase mitochondrion exclusively 5739 664215 3.1.2.1 acetyl-CoA hydrolase mitochondrion most of the Ach1p activity is distributed with mitochondria and little in the cytoplasm 5739 652385 3.1.2.11 acetoacetyl-CoA hydrolase mitochondrion - 5739 -, 5360, 80971, 80976, 80977 3.1.2.18 ADP-dependent short-chain-acyl-CoA hydrolase mitochondrion - 5739 646111, 646112, 646114, 646115 3.1.2.18 ADP-dependent short-chain-acyl-CoA hydrolase mitochondrion liver enzyme is mainly located in the light-mitochondrial fraction 5739 94490 3.1.2.19 ADP-dependent medium-chain-acyl-CoA hydrolase mitochondrion - 5739 646112 3.1.2.2 palmitoyl-CoA hydrolase mitochondrion - 5739 646115, 646121, 646125, 646126, 646128, 646131, 646138, 646140, 646144, 646149, 646150, 646151, 646155, 646156, 646158, 646162, 646163, 646171, 646173, 646180, 646187, 646190, 646192, 679724, 681262, 751271, 80783, 80784, 80787, 80791, 94478 3.1.2.2 palmitoyl-CoA hydrolase mitochondrion arachidonic acid-related thioesterase 5739 651003 3.1.2.2 palmitoyl-CoA hydrolase mitochondrion isozyme MTE I 5739 646163, 646188, 646197, 646203 3.1.2.2 palmitoyl-CoA hydrolase mitochondrion isozyme MTE-I 5739 646197, 651962 3.1.2.2 palmitoyl-CoA hydrolase mitochondrion matrix 5739 646112 3.1.2.2 palmitoyl-CoA hydrolase mitochondrion mitochondrial isozyme MTE-I 5739 651443 3.1.2.2 palmitoyl-CoA hydrolase mitochondrion several isoforms are differentially located in the subcellular compartments 5739 649607 3.1.2.2 palmitoyl-CoA hydrolase mitochondrion strongly decreased levels of ACOT7 activity and protein in mitochondria in patients diagnosed with fatty acid oxidation defects 5739 679133 3.1.2.20 acyl-CoA hydrolase mitochondrion - 5739 646115, 646121, 646125, 646126, 646128, 646131, 646138, 646140, 646144, 646149, 646150, 646151, 646155, 646156, 646158, 646162, 646163, 646171, 646173, 646180, 646187, 646190, 646192, 666795, 707368, 714050, 715623, 732503, 80783, 80784, 80787, 80791, 94478 3.1.2.20 acyl-CoA hydrolase mitochondrion isozyme MTE I 5739 646163, 646188, 646197, 646203 3.1.2.20 acyl-CoA hydrolase mitochondrion matrix 5739 646112 3.1.2.27 choloyl-CoA hydrolase mitochondrion - 5739 660497 3.1.2.27 choloyl-CoA hydrolase mitochondrion especially in the light mitochondrial fraction 5739 659661 3.1.2.29 fluoroacetyl-CoA thioesterase mitochondrion - 5739 712861 3.1.2.4 3-hydroxyisobutyryl-CoA hydrolase mitochondrion - 5739 653127, 94558 3.1.2.6 hydroxyacylglutathione hydrolase mitochondrion - 5739 134192, 134196, 652549, 663881, 680684, 692048, 692049, 692050, 750268 3.1.2.6 hydroxyacylglutathione hydrolase mitochondrion 10-15% of the total glyoxalase II activity in liver. 56% of the activity resides in the matrix, 19% in the intermembrane space. 3 isoforms are exclusively mitochondrial 5739 134177 3.1.2.6 hydroxyacylglutathione hydrolase mitochondrion at least 4 enzyme forms, contain 10% of the total glyoxalase II activity in the homogenate 5739 134187 3.1.2.6 hydroxyacylglutathione hydrolase mitochondrion intermembrane space and matrix 5739 134186 3.1.2.6 hydroxyacylglutathione hydrolase mitochondrion isoform GLYII-6 and GLYII-13 5739 750671 3.1.2.6 hydroxyacylglutathione hydrolase mitochondrion isoform glyoxalase II 5739 665680 3.1.2.6 hydroxyacylglutathione hydrolase mitochondrion mitochondrial isozyme 5739 653833 3.1.21.10 crossover junction endodeoxyribonuclease mitochondrion - 5739 134319, 134330, 650985, 653238 3.1.22.1 deoxyribonuclease II mitochondrion fraction of procaspase-8 and LEI colocalise in the mitochondria. After etoposide treatment, cathepsin D is released from lysosomes and part of it colocalises with caspase-8 and LEI 5739 696403 3.1.26.11 tRNase Z mitochondrion - 5739 -, 441609, 441612, 441615, 441621, 441622, 730705, 730934, 730939, 751507 3.1.26.11 tRNase Z mitochondrion dual localization of ELAC2 in the nucleus and mitochondrion 5739 750419 3.1.26.11 tRNase Z mitochondrion isoform Trz2 5739 -, 730021 3.1.26.11 tRNase Z mitochondrion isozyme ELAC2 5739 716895 3.1.26.11 tRNase Z mitochondrion isozyme RNase ZL, alternative translation initiation is responsible for the dual targeting of RNase ZL 5739 716704 3.1.26.11 tRNase Z mitochondrion isozyme SpTrz2p, is a mitochondrial protein with an N-terminal MTS,mitochondrial-targeting signal 5739 714095 3.1.26.11 tRNase Z mitochondrion TrZL2 5739 710310 3.1.26.3 ribonuclease III mitochondrion - 5739 -, 716905, 751633 3.1.26.4 ribonuclease H mitochondrion - 5739 134068, 714345, 729704, 750633, 751754, 751911 3.1.26.4 ribonuclease H mitochondrion in the cytoplasm, RNase H1 is enriched in the mitochondria 5739 751620 3.1.26.4 ribonuclease H mitochondrion mitochondrial RNase H1 5739 716211 3.1.26.4 ribonuclease H mitochondrion the enzyme contains a mitochondrial targeting sequence with the first 40 amino acid residues at the N-terminus, the enzyme is imported into mitochondrial vesicles in vitro, the enzyme is processed after entry into mitochondria 5739 666288 3.1.26.5 ribonuclease P mitochondrion - 5739 -, 134437, 134443, 134456, 654201, 656107, 682838, 691665, 691666, 708512, 709859, 710127, 710153, 710543, 710544, 730931, 751108, 751861 3.1.26.5 ribonuclease P mitochondrion all putative components of the enzyme are encoded by the nucleus 5739 134462 3.1.26.5 ribonuclease P mitochondrion isozyme PRORP2 5739 730931 3.1.26.5 ribonuclease P mitochondrion mitochondrial enzyme form of RNase MRP 5739 656352 3.1.26.5 ribonuclease P mitochondrion mitochondrial ribozyme form 5739 656352 3.1.26.5 ribonuclease P mitochondrion mtRNase P 5739 656743 3.1.26.5 ribonuclease P mitochondrion PRORP1 5739 716314, 730931 3.1.26.5 ribonuclease P mitochondrion RNase P and RNase MRP 5739 657340 3.1.26.5 ribonuclease P mitochondrion RNases P and MRP 5739 654201 3.1.26.5 ribonuclease P mitochondrion RNases P and MRP, enzyme consists of RNA component Rpm1r and protein component Rpm2p, the latter is required for the processing of the first 5739 654201 3.1.26.5 ribonuclease P mitochondrion the enzyme is composed of two proteins, protein PRORP2 is localized to the branched trypanosomatid mitochondrion and has a cleavable N-terminal targeting sequence 5739 729548 3.1.26.5 ribonuclease P mitochondrion the RNA subunits of the mitochondrial and nuclear enzymes are identical 5739 656107 3.1.3.102 FMN hydrolase mitochondrion intermembrane space 5739 209924 3.1.3.106 2-lysophosphatidate phosphatase mitochondrion - 5739 754054 3.1.3.108 nocturnin mitochondrion - 5739 754825 3.1.3.108 nocturnin mitochondrion based on the presence of a mitochondrial target sequence in NOCT, it is determined that mouse NOCT protein localizes to the mitochondria 5739 757298 3.1.3.108 nocturnin mitochondrion mitochondrial form of nocturnin possesses high-amplitude circadian rhythmicity with peak expression level during the early dark phase 5739 758253 3.1.3.11 fructose-bisphosphatase mitochondrion - 5739 715750, 715752 3.1.3.16 protein-serine/threonine phosphatase mitochondrion - 5739 134616, 134617, 134655, 680236, 94882 3.1.3.24 sucrose-phosphate phosphatase mitochondrion - 5739 114204 3.1.3.27 phosphatidylglycerophosphatase mitochondrion - 5739 749968 3.1.3.32 polynucleotide 3'-phosphatase mitochondrion the enzyme contains a functional mitochondrial-targeting signal, consisting of amino acids 432-441 (ARYVQCARAA), close to its C-terminus 5739 723293 3.1.3.34 deoxynucleotide 3'-phosphatase mitochondrion - 5739 678246 3.1.3.4 phosphatidate phosphatase mitochondrion - 5739 134862, 134876, 134879, 134892, 677543, 715035 3.1.3.4 phosphatidate phosphatase mitochondrion 45-kDA enzyme form 5739 134878 3.1.3.4 phosphatidate phosphatase mitochondrion the Mg2+-independent enzyme is mainly located in plasma membrane, mitochondria and microsomes 5739 134882 3.1.3.43 [pyruvate dehydrogenase (acetyl-transferring)]-phosphatase mitochondrion - 5739 -, 668737, 673647, 677467, 687812, 695493, 698173, 698844, 701553, 730051, 730466, 750487, 751092, 751248, 751678, 94881, 94882, 94883, 94884, 94886, 94888, 94889, 94890, 94891, 94892, 94893, 94894, 94895, 94898, 94899, 94901, 94904, 94906, 94907, 94908, 94909, 94910, 94911, 94912, 94913, 94914, 94916, 94917, 94919, 94920, 94921 3.1.3.51 dolichyl-phosphatase mitochondrion loosely bound to membrane 5739 134919 3.1.3.52 [3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)]-phosphatase mitochondrion - 5739 134655, 643855, 643857, 643859, 643860, 643861, 751367 3.1.3.52 [3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)]-phosphatase mitochondrion inner membrane-matrix compartment 5739 643856 3.1.3.59 alkylacetylglycerophosphatase mitochondrion 28% of the total activity 5739 646418 3.1.3.59 alkylacetylglycerophosphatase mitochondrion 40% of the total activity 5739 646420 3.1.30.1 Aspergillus nuclease S1 mitochondrion - 5739 134991, 134999 3.1.30.2 Serratia marcescens nuclease mitochondrion - 5739 135010, 678425 3.1.4.11 phosphoinositide phospholipase C mitochondrion isoform PLC1 5739 749956 3.1.4.12 sphingomyelin phosphodiesterase mitochondrion - 5739 135155, 135170, 135173, 704668 3.1.4.3 phospholipase C mitochondrion - 5739 730853 3.1.4.37 2',3'-cyclic-nucleotide 3'-phosphodiesterase mitochondrion - 5739 135396, 135408, 682134, 749958, 750868 3.1.4.37 2',3'-cyclic-nucleotide 3'-phosphodiesterase mitochondrion distribution of isozymes in mitochondrial fractions, i.e. myelin fraction, synaptic and nonsynaptic mitochondria, obtained after separation of brain mitochondria 5739 -, 729185 3.1.4.37 2',3'-cyclic-nucleotide 3'-phosphodiesterase mitochondrion enzyme contains a mitochondrial targeting signal at the N-terminus. Import to mitochondria leads to cleavage of signal sequence yielding a mature, truncated form of CNP2 similar in size as CNP1. CNP2 is localized specifically to mitochondria in non-myelinating cells, e.g. in adult liver or embryonic brain. Phosphorylation of the signal sequence by protein kinase C inhibits translocation to mitochondria. 5739 670268 3.1.4.37 2',3'-cyclic-nucleotide 3'-phosphodiesterase mitochondrion isoform II, intermembrane space 5739 730475 3.1.4.37 2',3'-cyclic-nucleotide 3'-phosphodiesterase mitochondrion the enzyme is associated with complexes I-V in mitochondria 5739 751698 3.1.4.37 2',3'-cyclic-nucleotide 3'-phosphodiesterase mitochondrion the N-terminus ofisozyme CNP2 serves as a mitochondrial targeting signal and translocates it to the mitochondrion 5739 730760 3.1.4.4 phospholipase D mitochondrion - 5739 -, 666655, 709222 3.1.4.40 CMP-N-acylneuraminate phosphodiesterase mitochondrion - 5739 135477 3.1.4.44 glycerophosphoinositol glycerophosphodiesterase mitochondrion - 5739 679873 3.1.4.49 dolichylphosphate-mannose phosphodiesterase mitochondrion - 5739 646471 3.1.6.2 steryl-sulfatase mitochondrion - 5739 135595 3.2.1.143 poly(ADP-ribose) glycohydrolase mitochondrion isoform hPARG55, strictly targeted to mitochondria. Isoform hPARG may be capable of shuttling between nucleus and mitochondria 5739 679683 3.2.1.143 poly(ADP-ribose) glycohydrolase mitochondrion PARG59 expression constructs with amino acids encoded by exon 4 at the N-terminus are targeted to the mitochondrial matrix 5739 708414 3.2.1.17 lysozyme mitochondrion - 5739 171054 3.2.1.18 exo-alpha-sialidase mitochondrion - 5739 679866, 702648, 729760 3.2.1.22 alpha-galactosidase mitochondrion - 5739 171187 3.2.1.28 alpha,alpha-trehalase mitochondrion alkaline trehalase 5739 665118 3.2.2.15 DNA-deoxyinosine glycosylase mitochondrion - 5739 656693 3.2.2.19 [protein ADP-ribosylarginine] hydrolase mitochondrion isoform ARH3 5739 731708 3.2.2.21 DNA-3-methyladenine glycosylase II mitochondrion - 5739 646893 3.2.2.23 DNA-formamidopyrimidine glycosylase mitochondrion - 5739 709080, 732941 3.2.2.27 uracil-DNA glycosylase mitochondrion - 5739 710138 3.2.2.27 uracil-DNA glycosylase mitochondrion isozyme UNG1 5739 710229 3.2.2.27 uracil-DNA glycosylase mitochondrion the hUNG1 isoform bears a mitochondrial leader peptide on its N-terminus and is imported into mitochondria 5739 710029 3.2.2.5 NAD+ glycohydrolase mitochondrion - 5739 137081, 137088, 137089, 137095 3.2.2.6 ADP-ribosyl cyclase/cyclic ADP-ribose hydrolase mitochondrion mitochondrial membrane 5739 732501 3.3.2.10 soluble epoxide hydrolase mitochondrion - 5739 683095, 699685, 731877 3.3.2.2 lysoplasmalogenase mitochondrion - 5739 137152, 137154 3.3.2.2 lysoplasmalogenase mitochondrion 14% of the activity 5739 137153, 137154 3.3.2.9 microsomal epoxide hydrolase mitochondrion - 5739 699685 3.3.2.9 microsomal epoxide hydrolase mitochondrion very low activity 5739 208899 3.4.11.1 leucyl aminopeptidase mitochondrion two mitochondrial derivatives differentiate and by the end of spermatogenesis the minor one reduces its size and the major one accumulates paracrystalline material inside it. The S-Lap enzymes are important constituents of the paracrystalline material in mitochondria of Drosophila melanogaster sperm. S-Lap proteins localize and accumulate in the paracrystalline material of the major mitochondrial derivative, S-Lap proteins are collectively present in the isolates 5739 755065 3.4.11.14 cytosol alanyl aminopeptidase mitochondrion - 5739 666460 3.4.11.18 methionyl aminopeptidase mitochondrion - 5739 678232 3.4.11.26 intermediate cleaving peptidase 55 mitochondrion - 5739 709066, 732385, 755046 3.4.11.26 intermediate cleaving peptidase 55 mitochondrion Icp55 behaves like a protein that is peripherally attached to the mitochondrial inner membrane from the matrix side 5739 708076 3.4.11.26 intermediate cleaving peptidase 55 mitochondrion transcript ICP55.1 is localized in the mitochondrion 5739 754294 3.4.11.3 cystinyl aminopeptidase mitochondrion - 5739 35902, 35918 3.4.11.3 cystinyl aminopeptidase mitochondrion low level of enzyme 5739 683795 3.4.11.7 glutamyl aminopeptidase mitochondrion - 5739 666460 3.4.11.9 Xaa-Pro aminopeptidase mitochondrion - 5739 709273 3.4.17.21 Glutamate carboxypeptidase II mitochondrion associated with 5739 137230 3.4.21.105 rhomboid protease mitochondrion - 5739 659229, 665042, 710565, 710566, 732070 3.4.21.105 rhomboid protease mitochondrion inner membrane 5739 659997 3.4.21.105 rhomboid protease mitochondrion intramembrane enzyme 5739 658507 3.4.21.105 rhomboid protease mitochondrion isoform PARL 5739 718193 3.4.21.105 rhomboid protease mitochondrion protease is localised only in the inner mitochondrial membrane, with the N-terminus exposed to the matrix and the C-terminus to the IMS 5739 710566 3.4.21.105 rhomboid protease mitochondrion Rbd1 and Ygr101w 5739 683499 3.4.21.107 peptidase Do mitochondrion - 5739 653904 3.4.21.107 peptidase Do mitochondrion isoform HTRA2 5739 718153 3.4.21.108 HtrA2 peptidase mitochondrion - 5739 652327, 652366, 653342, 667167, 667405, 667757, 668267, 668700, 669260, 669278, 669282, 687539, 689124, 689170, 689287, 689289, 695311, 695598, 697172, 697176, 697488, 697749, 698185, 699486, 699689, 700569, 700912, 700949, 708821, 709955, 710093, 752464, 752820, 753188, 753695, 755509 3.4.21.108 HtrA2 peptidase mitochondrion amyloid precursor protein is partly colocalized with HtrA2 in the mitochondria 5739 687526 3.4.21.108 HtrA2 peptidase mitochondrion extramitochondrially expressed enzyme induces atypical cell death 5739 653199 3.4.21.108 HtrA2 peptidase mitochondrion healthy cell 5739 697174 3.4.21.108 HtrA2 peptidase mitochondrion HtrA2 in the intermembrane space of healthy cells, density of mitochondria decreased and showed abnormal forms in knockout mice 5739 670241 3.4.21.108 HtrA2 peptidase mitochondrion HtrA2 is a mitochondrial protease 5739 713031 3.4.21.108 HtrA2 peptidase mitochondrion immature protein 5739 732873 3.4.21.108 HtrA2 peptidase mitochondrion immunostaining and confocal microscopy shows that Omi/HtrA2 is located primarily in the mitochondria of lung adenocarcinoma cell line A549 5739 709872 3.4.21.108 HtrA2 peptidase mitochondrion in response to UV irradiation the enzyme shifts into the cytosol 5739 652331 3.4.21.108 HtrA2 peptidase mitochondrion intermembrane space 5739 668190 3.4.21.108 HtrA2 peptidase mitochondrion intermembrane space, HtrA2/Omi is required for mitochondrial homeostasis 5739 686002 3.4.21.108 HtrA2 peptidase mitochondrion mainly localized 5739 670224 3.4.21.108 HtrA2 peptidase mitochondrion mature protein is released form the mitochondria into the cytosol during aopotosis 5739 652439 3.4.21.108 HtrA2 peptidase mitochondrion mitochondria also contain processed Omi which, following apoptotic insult, translocates to the cytosol 5739 652329 3.4.21.108 HtrA2 peptidase mitochondrion normally expression of HtrA2, enzyme does not leave the mitochondria upon the TNF-alpha + zVAD-induced cell death in neutrophils 5739 668191 3.4.21.108 HtrA2 peptidase mitochondrion Omi/HtrA2 and Smac/Diablo are released from mitochondria into the platelet cytosol together with cytochrome c after induction of apoptosis by theCa2+ ionophore A23187 or the BH3 mimetic ABT-737, and to a lesser extent, after platelet stimulation with collagen and thrombin 5739 732929 3.4.21.108 HtrA2 peptidase mitochondrion predominant localisation, enlarged mitochondria with disorganized cristae in mutant cells 5739 668876 3.4.21.108 HtrA2 peptidase mitochondrion predominant presence of Omi 5739 -, 667167 3.4.21.108 HtrA2 peptidase mitochondrion released into cytosol during apoptosis 5739 649619 3.4.21.108 HtrA2 peptidase mitochondrion released into the cytosol upon apoptotic stimuli 5739 697363 3.4.21.108 HtrA2 peptidase mitochondrion S-nitrosoglutathione treatment induces redistribution of Omi/HtrA2 from cytoplasm to mitochondria in endothelial cells 5739 713539 3.4.21.108 HtrA2 peptidase mitochondrion translocation from mitochondria to cytosol 5739 697154 3.4.21.26 prolyl oligopeptidase mitochondrion - 5739 692518 3.4.21.53 Endopeptidase La mitochondrion - 5739 -, 29341, 29355, 29356, 29357, 650959, 651411, 651835, 651846, 651853, 651889, 653293, 653612, 653632, 653881, 663454, 669354, 677574, 678475, 680779, 681850, 681937, 682726, 682821, 697889, 710500, 717017, 718095, 718319, 731218, 731618, 731762, 731835, 732469, 752803, 752805, 753692, 753718 3.4.21.53 Endopeptidase La mitochondrion human Lon protease localizes to mitochondrial nucleoids 5739 755478 3.4.21.53 Endopeptidase La mitochondrion in isolated nonsynaptic brain mitochondria Lon protease is highly susceptible to oxidative inactivation by peroxynitrite. This susceptibility is more pronounced with regard to ATP-stimulated activity, which is inhibited by 75% in the presence of a bolus addition of 1 mM ONOO, whereas basal unstimulated activity is inhibited by 45%. A decline in Lon protease activity precedes electron transport chain dysfunction and ATP-stimulated activity is approximately fivefold more sensitive than basal Lon protease activity. Supplementation of mitochondrial matrix extracts with reduced glutathione, following peroxynitrite exposure, results in partial restoration of basal and ATP-stimulated activity 5739 700423 3.4.21.53 Endopeptidase La mitochondrion lon1, lon3 and lon4 5739 682349 3.4.21.53 Endopeptidase La mitochondrion matrix 5739 677574 3.4.21.53 Endopeptidase La mitochondrion mitochondrial ATP-dependent proteolytic activity is reduced by 35% in Deltapim1 cells. Oxidized proteins accumulate at elevated levels in mitochondria lacking pim1 activity 5739 709088 3.4.21.53 Endopeptidase La mitochondrion mitochondrial Lon protease contains a mitochondrial targeting sequence 5739 755444 3.4.21.53 Endopeptidase La mitochondrion mitochondrial matrix 5739 651419 3.4.21.53 Endopeptidase La mitochondrion prepared from 4days old epicotyls of Zea mays L. seedlings 5739 685483 3.4.21.53 Endopeptidase La mitochondrion yeast Lon protease localizes to mitochondrial nucleoids 5739 -, 755478 3.4.21.78 granzyme A mitochondrion - 5739 697233 3.4.21.89 Signal peptidase I mitochondrion inner membrane 5739 29813 3.4.21.89 Signal peptidase I mitochondrion mitochondrial inner membrane 5739 653816 3.4.21.92 Endopeptidase Clp mitochondrion - 5739 653260, 697889, 700468, 752813 3.4.21.B25 PACE4 proprotein convertase mitochondrion in stage 1 oocytes, XPACE4 localizes to the mitochondrial cloud, at stage 2, the mitochondrial cloud disperses, then XPACE4 mRNA co-localizes with the vegetal cortex where it remains for the rest of oogenesis 5739 673252 3.4.21.B59 granzyme H mitochondrion - 5739 732517 3.4.22.1 cathepsin B mitochondrion - 5739 753138 3.4.22.52 calpain-1 mitochondrion - 5739 708761, 710069, 753389 3.4.22.52 calpain-1 mitochondrion a pool of micro-calpain is localized to the mitochondrial intermembrane space 5739 708435 3.4.22.52 calpain-1 mitochondrion association between l-calpain and calpastatin in the mitochondria 5739 677829 3.4.22.52 calpain-1 mitochondrion both calpain 1 and calpain small subunit 1, which together form mu-calpain, are present in the mitochondrial intermembrane space. The N-terminus of calpain 1 is not processed following mitochondrial import, but is removed by autolysis following calpain activation. Calpain small subunit 1 is not directly imported into mitochondria, but is imported in the presence of calpain 1. The presence of a mitochondrial targeting sequence in the N-terminal region of calpain 1 is consistent with the localization of mu-calpain to the mitochondrial intermembrane space 5739 680913 3.4.22.52 calpain-1 mitochondrion mitochondrial calpain 1 is located within the intermembrane space and mitochondrial matrix 5739 752801 3.4.22.52 calpain-1 mitochondrion mu-calpain is present in the inner mitochondrial membrane, but not in the outer mitochondrial membrane or in the inter membrane space or in the matrix of the mitochondria. mu-Calpain-calpastatin is associated in the inner mitochondrial membrane. mu-Calpain is integrally and calpastatin is peripherally embedded to the outer surface of inner mitochondrial membrane 5739 677835 3.4.22.52 calpain-1 mitochondrion mu-calpain, including both the large and small subunits is present in the mitochondrial intermembrane space 5739 707281 3.4.22.52 calpain-1 mitochondrion present mostly in the intermembrane space 5739 717201 3.4.22.53 calpain-2 mitochondrion - 5739 707559, 717157 3.4.22.53 calpain-2 mitochondrion m-calpain is present in the intermembrane space of the mitochondria 5739 707281 3.4.22.55 caspase-2 mitochondrion - 5739 -, 663875, 664286, 664533, 665553, 665588, 666298, 753185 3.4.22.55 caspase-2 mitochondrion shift of caspase 2 from the cytoplasm in the sham-operated testis to the mitochondria in the testis after torsion 5739 679114 3.4.22.56 caspase-3 mitochondrion - 5739 647420, 700685, 717470, 718432 3.4.22.61 caspase-8 mitochondrion - 5739 664711, 664969, 666402, 666515, 717470 3.4.22.61 caspase-8 mitochondrion the mitochondrial membrane protein Cardif plays a role in caspase-8 translocation to mitochondria. The anionic mitochondria-specific phospho-lipid cardiolipin acts as a mitochondria-associated platform that is actually required for caspase-8 translocation in type II cells 5739 717276 3.4.22.62 caspase-9 mitochondrion - 5739 647420, 663674, 717470, 718432 3.4.22.B25 calpain 5 mitochondrion calpain-5 is localized in the mitochondria of photoreceptor cells and is present in each mitochondrial subfraction. The molecular weight of mitochondrial calpain-5 is slightly smaller than the cytosolic one 5739 752685 3.4.22.B30 calpain 10 mitochondrion - 5739 690381, 718063 3.4.22.B30 calpain 10 mitochondrion matrix 5739 693580 3.4.22.B30 calpain 10 mitochondrion mitochondrial calpain 10 is required for cell viability 5739 690381 3.4.22.B30 calpain 10 mitochondrion mitochondrial outer membrane, intermembrane space, inner membrane, matrix 5739 663594 3.4.22.B30 calpain 10 mitochondrion several splice variants of calpain 10 are localized to the mitochondria in kidney 5739 677984 3.4.22.B73 SENP5 peptidase mitochondrion in interphase, SenP5 resides primarily within the nucleoli, in addition to a cytosolic pool. Relocalization of SenP5 from the nucleoli to the mitochondrial surface at G2/M transition prior to nuclear envelope breakdown. Transition of SenP5 to the mitochondria plays an important role in mitochondrial fragmentation during mitosis 5739 715497 3.4.22.B73 SENP5 peptidase mitochondrion Senp5 is localized to presynaptic terminals, postsynaptic spines, and mitochondria in axon terminals 5739 754262 3.4.24.15 thimet oligopeptidase mitochondrion - 5739 753583 3.4.24.16 neurolysin mitochondrion - 5739 -, 37063, 37077, 668828, 754221, 754607, 754898 3.4.24.16 neurolysin mitochondrion EP24.16m is the mitochondrial enzyme form 5739 37061 3.4.24.16 neurolysin mitochondrion intermembrane space 5739 37085 3.4.24.16 neurolysin mitochondrion longer form of neurolysin that contains a cleavable mitochondrial targeting sequence at the N-terminus 5739 668828 3.4.24.16 neurolysin mitochondrion tagged full-length NLN (hNLN1-704) 5739 754429 3.4.24.24 gelatinase A mitochondrion - 5739 752865 3.4.24.37 saccharolysin mitochondrion intermembrane space 5739 668761, 669339 3.4.24.56 insulysin mitochondrion - 5739 710682, 755096 3.4.24.56 insulysin mitochondrion a form of the enzyme derived from an alternative translational start site that can localize to mitochondria 5739 683342 3.4.24.56 insulysin mitochondrion isoform generated by translation of enzyme at an in-frame initiation codon 123 nucleotides upstream of the canonical translation start site, resulting in addition of a 41 amino acid N-terminal mitochondrial targeting sequence 5739 667484 3.4.24.56 insulysin mitochondrion isoform IDE-15b, both in cytosol and mitochondrion 5739 667669 3.4.24.59 mitochondrial intermediate peptidase mitochondrion - 5739 668830, 683116, 733399, 734387, 753580, 755046 3.4.24.59 mitochondrial intermediate peptidase mitochondrion after import of MIP in mitochondrion, processing of the precursor to the mature form by MPP 5739 668830 3.4.24.59 mitochondrial intermediate peptidase mitochondrion the enzyme displays an exclusively mitochondrial localization 5739 755173 3.4.24.64 mitochondrial processing peptidase mitochondrion - 5739 -, 31314, 31382, 31386, 31387, 31392, 31395, 31398, 31399, 31400, 31401, 31403, 31408, 649576, 651890, 651893, 651907, 651908, 652393, 669838, 669840, 683617, 708076, 709066, 710508, 733381, 733730, 734853 3.4.24.64 mitochondrial processing peptidase mitochondrion 2 components, proteolytic activity bearing, matrix-soluble MPP protein and activating processing enhancing protein which is partly associated with the inner surface of the inner membrane 5739 31388 3.4.24.64 mitochondrial processing peptidase mitochondrion alpha-MPP: soluble 5739 31406 3.4.24.64 mitochondrial processing peptidase mitochondrion beta-MPP: membrane-associated 5739 31402, 31406 3.4.24.64 mitochondrial processing peptidase mitochondrion integral part of bc1-complex of respiratory chain 5739 31404 3.4.24.64 mitochondrial processing peptidase mitochondrion intermembrane space 5739 683860 3.4.24.64 mitochondrial processing peptidase mitochondrion matrix 5739 -, 31383, 31384, 31385, 31388, 31389, 31390, 31391, 31393, 31394, 31396, 31405, 651520, 651746 3.4.24.64 mitochondrial processing peptidase mitochondrion mitochondrial matrix space 5739 683664, 683686 3.4.24.64 mitochondrial processing peptidase mitochondrion protein complex of the inner mitochondrial membrane 5739 651520 3.4.24.64 mitochondrial processing peptidase mitochondrion subcellular localization study, overview 5739 709853 3.4.24.7 interstitial collagenase mitochondrion intracellular association of MMP-1 to mitochondria and nuclei confers resistance to apoptosis and may explain the association of this enzyme with tumor cell survival and spreading 5739 667138 3.4.24.B18 m-AAA protease mitochondrion - 5739 -, 649814, 650372, 650958, 651889, 653198, 668180, 668412, 679501, 681464, 681833, 692577, 718997, 719365, 719994, 733797, 734573, 734690, 752423, 754149, 754180 3.4.24.B18 m-AAA protease mitochondrion active on the matrix side of the inner membrane 5739 682956 3.4.24.B18 m-AAA protease mitochondrion human mitochondria possess two m-AAA protease isoenzymes: a hetero-oligomeric complex, composed of paraplegin and AFG3L2 (Afg3 like 2), and a homo-oligomeric AFG3L2 complex. Genetic interaction between the m-AAA isoenzymes and suggest that different neuronal populations have variable thresholds of susceptibility to reduced levels of the m-AAA protease 5739 692577 3.4.24.B18 m-AAA protease mitochondrion inner mitochondrial membrane 5739 732470, 733724 3.4.24.B18 m-AAA protease mitochondrion mitochondrial inner membrane 5739 753920 3.4.24.B19 i-AAA protease mitochondrion - 5739 -, 649814, 650958, 653335, 670047, 681914, 734658, 753423, 754234 3.4.24.B19 i-AAA protease mitochondrion inner mitochondrial membrane, oriented toward the intermembrane space 5739 719236 3.4.24.B19 i-AAA protease mitochondrion mitochondrial intermembrane space 5739 -, 753035 3.4.24.B19 i-AAA protease mitochondrion stomatin-like protein localizes to the mitochondrial inner membrane and anchors a large protease complex composed of the rhomboid protease PARL and the i-AAA protease YME1L. Association with SLP2 in the SPY complex regulates PARL-mediated processing of PTEN-induced kinase PINK1 and the phosphatase PGAM5 in mitochondria 5739 753424 3.4.24.B19 i-AAA protease mitochondrion tightly associated with the inner mitochondrial membrane, oriented with the bulk of the protein facing the matrix 5739 653183 3.4.24.B19 i-AAA protease mitochondrion Yme1p is associated with the Mgr1 protein in mitochondria 5739 670211 3.4.24.B20 FtsH protease mitochondrion - 5739 668355, 670580, 712664, 713211, 753102 3.4.24.B20 FtsH protease mitochondrion Arabidopsis thalaina contains 12 genes encoding FtsH proteins, nine of them can be targeted to chloroplast, the other three are mitochondrial 5739 667208 3.4.24.B20 FtsH protease mitochondrion inner mitochondrial membrane 5739 735098 3.4.24.B20 FtsH protease mitochondrion isoforms FtsH3, 4 and 10 5739 734540 3.5.1.12 biotinidase mitochondrion - 5739 171922, 171933 3.5.1.12 biotinidase mitochondrion splice variants 1a and 1b localize to mitochondria and/or endoplasmic reticulum 5739 670277 3.5.1.13 aryl-acylamidase mitochondrion low activity of enzyme form AAA-1 5739 208974 3.5.1.13 aryl-acylamidase mitochondrion outer membrane 5739 208972 3.5.1.19 nicotinamidase mitochondrion - 5739 246568 3.5.1.2 glutaminase mitochondrion - 5739 209009, 209013, 209014, 209015, 209018, 209019, 209028, 209029, 209030, 209031, 655542, 657262, 699661, 700394, 713428, 718889 3.5.1.2 glutaminase mitochondrion GAC is mitochondrial, distinctly localized from kidney-type GLS isozymes 5739 720930 3.5.1.2 glutaminase mitochondrion isozyme KGA 5739 669592 3.5.1.2 glutaminase mitochondrion isozyme LGA 5739 670391 3.5.1.2 glutaminase mitochondrion loosely bound to inner membrane 5739 209017, 209027 3.5.1.2 glutaminase mitochondrion mitochondrial enzyme with a cytosolic precursor 5739 209034 3.5.1.2 glutaminase mitochondrion mitochondrial glutaminase plays a role in HIV-infected monocyte-derived macrophage-mediated neuronal apoptosis 5739 688517 3.5.1.2 glutaminase mitochondrion neuroblastomal PAG is mitochondrial, enriched in mitochondria by 606% compared to the cell homogenate 5739 713112 3.5.1.2 glutaminase mitochondrion phosphate-activated isozyme 5739 669694 3.5.1.2 glutaminase mitochondrion synaptosomal and non-synaptosomal 5739 209012 3.5.1.20 citrullinase mitochondrion - 5739 171969 3.5.1.23 ceramidase mitochondrion - 5739 -, 654614, 711710, 715623, 752416 3.5.1.3 omega-amidase mitochondrion - 5739 288884, 288891, 288893 3.5.1.3 omega-amidase mitochondrion 75% of omega-amidase is present in cytoplasm, only 25% of omega-amidase activity is associated with mitochondrial fraction 5739 288894 3.5.1.60 N-(long-chain-acyl)ethanolamine deacylase mitochondrion - 5739 209214 3.5.1.68 N-formylglutamate deformylase mitochondrion about 70% of total enzyme activity located in mitochondrial fractions 5739 -, 209222 3.5.1.75 urethanase mitochondrion moderate activity 5739 209238 3.5.1.88 peptide deformylase mitochondrion - 5739 654721, 675061, 687836, 688243, 689043, 699575, 700712, 713456, 718759, 733007, 733571, 756536, 757712 3.5.1.88 peptide deformylase mitochondrion AtDEF1.1 5739 689452 3.5.1.92 pantetheine hydrolase mitochondrion - 5739 757538 3.5.1.98 histone deacetylase mitochondrion isozyme GmHDA13 5739 756309 3.5.1.99 fatty acid amide hydrolase mitochondrion - 5739 718570 3.5.1.B18 4-aminobenzoylglutamate hydrolase mitochondrion - 5739 683975 3.5.2.5 allantoinase mitochondrion - 5739 246667, 246671 3.5.3.1 arginase mitochondrion - 5739 172101, 172112, 654333, 654612, 667815, 687215, 697211, 700806, 703632, 711094, 733483, 733678 3.5.3.1 arginase mitochondrion arginase II 5739 699995 3.5.3.1 arginase mitochondrion constitutive expression of enzyme 5739 670000 3.5.3.1 arginase mitochondrion membrane-bound 5739 754679 3.5.3.1 arginase mitochondrion of myocyte 5739 670700 3.5.3.11 agmatinase mitochondrion - 5739 246682 3.5.3.18 dimethylargininase mitochondrion conventional immunofluorescence and confocal microscopy reveal the presence of DDAH-2 in the mitochondria of IL-1beta-stimulated chondrocytes. Blocking the translocation to mitochondria reduces the NO production induced by IL-1beta 5739 718718 3.5.3.7 guanidinobutyrase mitochondrion - 5739 209474 3.5.4.12 dCMP deaminase mitochondrion - 5739 734401 3.5.4.16 GTP cyclohydrolase I mitochondrion - 5739 -, 720858 3.5.4.3 guanine deaminase mitochondrion - 5739 -, 209589, 209596 3.5.4.9 methenyltetrahydrofolate cyclohydrolase mitochondrion - 5739 209728, 209731, 701402, 712483, 712978, 734262, 756334, 756337, 757773 3.6.1.1 inorganic diphosphatase mitochondrion - 5739 209772, 209828, 755906 3.6.1.1 inorganic diphosphatase mitochondrion isozyme PPa1, which contains an N-terminal mitochondrial target sequence with a putative cleavage site at Val31 5739 689658 3.6.1.1 inorganic diphosphatase mitochondrion isozyme PPase2 5739 668747 3.6.1.1 inorganic diphosphatase mitochondrion isozyme sPPase-II 5739 667527 3.6.1.1 inorganic diphosphatase mitochondrion soluble 5739 -, 655580 3.6.1.1 inorganic diphosphatase mitochondrion soluble and membrane bound 5739 209829 3.6.1.1 inorganic diphosphatase mitochondrion the N-terminal peptide of immature AtPPA1 is mostly disordered. It can be cleaved during maturation. The cleaved peptide is a mitochondrial targeting signal 5739 756011 3.6.1.1 inorganic diphosphatase mitochondrion the N-terminal peptide of immature MtPPA1 can be cleaved during maturation. The cleaved peptide is a mitochondrial targeting signal 5739 756011 3.6.1.11 exopolyphosphatase mitochondrion - 5739 668027, 668674, 685307, 687085, 697460 3.6.1.11 exopolyphosphatase mitochondrion PPN1 5739 -, 685297 3.6.1.11 exopolyphosphatase mitochondrion soluble fraction 5739 696170 3.6.1.12 dCTP diphosphatase mitochondrion - 5739 756417 3.6.1.13 ADP-ribose diphosphatase mitochondrion - 5739 656126 3.6.1.17 bis(5'-nucleosyl)-tetraphosphatase (asymmetrical) mitochondrion isolated mitochondria used 5739 209899 3.6.1.18 FAD diphosphatase mitochondrion - 5739 693166, 721117 3.6.1.2 trimetaphosphatase mitochondrion - 5739 209930, 209936 3.6.1.20 5'-acylphosphoadenosine hydrolase mitochondrion - 5739 209942 3.6.1.23 dUTP diphosphatase mitochondrion - 5739 667399 3.6.1.23 dUTP diphosphatase mitochondrion enzyme form DUT-M 5739 209988 3.6.1.25 triphosphatase mitochondrion - 5739 210000, 210001 3.6.1.29 bis(5'-adenosyl)-triphosphatase mitochondrion - 5739 209899, 718972 3.6.1.29 bis(5'-adenosyl)-triphosphatase mitochondrion matrix 5739 289071 3.6.1.5 apyrase mitochondrion - 5739 -, 733802 3.6.1.6 nucleoside diphosphate phosphatase mitochondrion - 5739 210216 3.6.1.60 diadenosine hexaphosphate hydrolase (AMP-forming) mitochondrion - 5739 714949 3.6.1.70 guanosine-5'-diphospho-5'-[DNA] diphosphatase mitochondrion isoforms carrying a 42-nt N-terminal mitochondrial targeting sequence are localized to mitochondria 5739 755241 3.6.1.71 adenosine-5'-diphospho-5'-[DNA] diphosphatase mitochondrion isoforms carrying a 42-nt N-terminal mitochondrial targeting sequence are localized to mitochondria 5739 755241 3.6.1.72 DNA-3'-diphospho-5'-guanosine diphosphatase mitochondrion isoforms carrying a 42-nt N-terminal mitochondrial targeting sequence are localized to mitochondria 5739 755241 3.6.4.10 non-chaperonin molecular chaperone ATPase mitochondrion - 5739 667512, 734233, 735005, 757583 3.6.4.10 non-chaperonin molecular chaperone ATPase mitochondrion subcellular real-time PCR expression analysis of the enzyme in transiently transfected Arabidospsis thailana mesophyll protoplasts 5739 735150 3.6.4.10 non-chaperonin molecular chaperone ATPase mitochondrion the chaperones mtHsp70 and mtHsp40 and their cofactor Mge1 are uniformly distributed throughout the single mitochondrial network 5739 734614 3.6.4.13 RNA helicase mitochondrion - 5739 713564, 734004 3.6.4.B7 RadA recombinase mitochondrion - 5739 757830 3.6.5.3 protein-synthesizing GTPase mitochondrion - 5739 667314, 667781, 669421 3.6.5.3 protein-synthesizing GTPase mitochondrion EF-G1mt is active on both bacterial and mitochondrial ribosomes 5739 670763 3.6.5.5 dynamin GTPase mitochondrion - 5739 668369, 686382, 687540, 687638, 689529, 697536 3.6.5.5 dynamin GTPase mitochondrion anchored to the inner membrane 5739 733396 3.6.5.5 dynamin GTPase mitochondrion mitochondria are the principal source of dynamin-2 in BL-3 cells 5739 698249 3.6.5.6 tubulin GTPase mitochondrion despite evidence that the mitochondrial proteome is derived from endosymbiotic bacteria, most mitochondria lack the expression of the gamma-tubulin homologue FtsZ, suggesting that gamma-tubulin may replace FtsZ function in mitochondria 5739 756504 3.7.1.5 acylpyruvate hydrolase mitochondrion - 5739 719974, 756006 3.9.1.3 phosphohistidine phosphatase mitochondrion - 5739 757649 4.1.1.102 phenacrylate decarboxylase mitochondrion - 5739 -, 749347 4.1.1.112 oxaloacetate decarboxylase mitochondrion - 5739 4270, 756006 4.1.1.112 oxaloacetate decarboxylase mitochondrion 14% of activity 5739 653877 4.1.1.112 oxaloacetate decarboxylase mitochondrion differs from cytoplasmic enzyme 5739 4271 4.1.1.15 glutamate decarboxylase mitochondrion external surface of mitochondrion 5739 682116 4.1.1.19 arginine decarboxylase mitochondrion highly if not exclusively associated with mitochondrial membrane 5739 4097 4.1.1.29 sulfinoalanine decarboxylase mitochondrion - 5739 4238 4.1.1.31 phosphoenolpyruvate carboxylase mitochondrion - 5739 -, 727387, 746700 4.1.1.31 phosphoenolpyruvate carboxylase mitochondrion plant-type and bacterial-type phosphoenolpyruvate carboxylase (PEPC) interact in vivo as a class-2 PEPC complex that associates with the surface of mitochondria 5739 728484 4.1.1.32 phosphoenolpyruvate carboxykinase (GTP) mitochondrion - 5739 4344, 4349, 4350, 4353, 4359, 4367, 667608, 678205, 679083, 702944, 704540, 727993, 748770 4.1.1.32 phosphoenolpyruvate carboxykinase (GTP) mitochondrion 2 enzyme forms exist in livers of embryos and young chicken, M1 and M2. 1 enzyme form exists in liver of adults, M 5739 4345 4.1.1.32 phosphoenolpyruvate carboxykinase (GTP) mitochondrion cytosolic and mitochondrial isoform of PEPCK, encoded by two different nuclear genes, transition to lactation do not alter the mitochondrial PEPCK expression 5739 653433 4.1.1.32 phosphoenolpyruvate carboxykinase (GTP) mitochondrion mitochondrial isoenzyme 5739 649936 4.1.1.32 phosphoenolpyruvate carboxykinase (GTP) mitochondrion mitochondrial isoform 5739 650021 4.1.1.40 hydroxypyruvate decarboxylase mitochondrion 75% of the enzyme activity 5739 4543 4.1.1.42 carnitine decarboxylase mitochondrion - 5739 4555 4.1.1.6 cis-aconitate decarboxylase mitochondrion - 5739 723665 4.1.1.6 cis-aconitate decarboxylase mitochondrion mitochondrial isozyme 5739 728240 4.1.1.65 phosphatidylserine decarboxylase mitochondrion - 5739 -, 661239, 680820, 680942, 682423, 682435, 693510, 694160, 715603, 727932, 727992, 728629, 748652, 748671 4.1.1.65 phosphatidylserine decarboxylase mitochondrion anchored to the inner mitochondrial membrane 5739 747186 4.1.1.65 phosphatidylserine decarboxylase mitochondrion autocatalytic processing in the mitochondrion 5739 748188 4.1.1.65 phosphatidylserine decarboxylase mitochondrion inner mitochondrial membrane, isoform Psd1p 5739 -, 702464 4.1.1.65 phosphatidylserine decarboxylase mitochondrion isoform PSD1 5739 -, 703770 4.1.1.65 phosphatidylserine decarboxylase mitochondrion membrane of mitochondria 5739 653541 4.1.1.65 phosphatidylserine decarboxylase mitochondrion PSD1 5739 694089, 705619 4.1.1.65 phosphatidylserine decarboxylase mitochondrion the 56 kDa precursor is processed upon import into mitochondria, leading to a 4 kDa alpha-subunit and a 47 kDa beta-subunit. Mutations in the conserved motif of the alpha-subunit affect processing and stability of Psd1, and consequently the enzyme's activity 5739 747185 4.1.1.65 phosphatidylserine decarboxylase mitochondrion the enzyme requires integration into the inner mitochondrial membrane for full enzymatic activity 5739 -, 727929 4.1.1.71 2-oxoglutarate decarboxylase mitochondrion exclusively 5739 648364 4.1.1.71 2-oxoglutarate decarboxylase mitochondrion synaptic and nonsynaptic 5739 349037 4.1.1.72 branched-chain-2-oxoacid decarboxylase mitochondrion - 5739 648371, 648376 4.1.1.9 malonyl-CoA decarboxylase mitochondrion - 5739 4844, 4849, 4862, 690446, 691684, 727260, 728812, 748540, 749255 4.1.1.9 malonyl-CoA decarboxylase mitochondrion 50% of MCD activity in heart and 65% in skeletal muscle is localized to mitochondria, while 50% and 35% is extramitochondrial 5739 649317 4.1.1.9 malonyl-CoA decarboxylase mitochondrion a small portion is present in mitochondria 5739 4854 4.1.1.9 malonyl-CoA decarboxylase mitochondrion enzyme from liver 5739 4858 4.1.1.9 malonyl-CoA decarboxylase mitochondrion enzyme may be targeted to mitochondria in addition to, or possibly instead of, peroxisomes 5739 652698 4.1.1.9 malonyl-CoA decarboxylase mitochondrion intramitochondrial distribution 5739 4843 4.1.1.9 malonyl-CoA decarboxylase mitochondrion matrix 5739 4854 4.1.1.9 malonyl-CoA decarboxylase mitochondrion matrix space 5739 4845 4.1.1.9 malonyl-CoA decarboxylase mitochondrion tightly bound 5739 4846 4.1.2.27 sphinganine-1-phosphate aldolase mitochondrion - 5739 682957 4.1.3.16 4-Hydroxy-2-oxoglutarate aldolase mitochondrion - 5739 5237, 713711 4.1.3.16 4-Hydroxy-2-oxoglutarate aldolase mitochondrion 89% of total activity 5739 5253 4.1.3.30 Methylisocitrate lyase mitochondrion - 5739 -, 5344, 714846 4.1.3.4 hydroxymethylglutaryl-CoA lyase mitochondrion - 5739 5363, 652699, 665743, 681950, 681959, 691869, 693782, 704083, 713966, 715807 4.1.3.4 hydroxymethylglutaryl-CoA lyase mitochondrion 3-hydroxy-3-methylglutaryl-CoA lyase 5739 727926 4.1.3.4 hydroxymethylglutaryl-CoA lyase mitochondrion 87% of activity 5739 5360 4.1.3.4 hydroxymethylglutaryl-CoA lyase mitochondrion exclusively 5739 5364 4.1.3.4 hydroxymethylglutaryl-CoA lyase mitochondrion matrix 5739 5362, 5364, 5372 4.1.99.12 3,4-dihydroxy-2-butanone-4-phosphate synthase mitochondrion in the intermembrane space, possibly associated with the outer membrane, minor enzyme portion 5739 674407 4.1.99.22 GTP 3',8-cyclase mitochondrion - 5739 -, 747034 4.1.99.3 deoxyribodipyrimidine photo-lyase mitochondrion - 5739 749375 4.1.99.3 deoxyribodipyrimidine photo-lyase mitochondrion contains a high proportion of unphosphorylated CPD photolyase 5739 -, 728501 4.1.99.3 deoxyribodipyrimidine photo-lyase mitochondrion CPD photolyase functions in mitochondria. Mitochondria show a high proportion of non-phosphorylated CPD photolyase 5739 716545 4.2.1.1 carbonic anhydrase mitochondrion - 5739 33571, 714606, 746566, 748948 4.2.1.1 carbonic anhydrase mitochondrion betaCA6 is targeted to the mitochondria 5739 682333 4.2.1.1 carbonic anhydrase mitochondrion contain N-terminal signal peptide for translocation into mitochondria 5739 729718 4.2.1.1 carbonic anhydrase mitochondrion isoforms CA VA and CA VB 5739 702654, 702679 4.2.1.107 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA hydratase mitochondrion - 5739 658438 4.2.1.11 phosphopyruvate hydratase mitochondrion up to 7% of enolase activity associated with mitochondria, targeting towards the mitochondrial outer membrane, affinity to the mitochondrial surface more than 10fold higher for Eno2p than for Eno1p 5739 680008 4.2.1.114 methanogen homoaconitase mitochondrion - 5739 -, 746978 4.2.1.136 ADP-dependent NAD(P)H-hydrate dehydratase mitochondrion - 5739 -, 728528 4.2.1.150 short-chain-enoyl-CoA hydratase mitochondrion - 5739 -, 748646 4.2.1.17 enoyl-CoA hydratase mitochondrion - 5739 33730, 33734, 5866, 649889, 650113, 651567, 689308, 697212, 697604, 697671, 697690, 699508, 730242, 746582, 746611, 747603, 748661 4.2.1.18 methylglutaconyl-CoA hydratase mitochondrion - 5739 679737, 681226, 748959 4.2.1.18 methylglutaconyl-CoA hydratase mitochondrion mitochondrial matrix 5739 -, 748625 4.2.1.2 fumarate hydratase mitochondrion - 5739 -, 33749, 33757, 33763, 33773, 653454, 677931, 681328, 696399, 700824, 703151, 705784, 706242, 706404, 715093, 729840, 730558, 746638, 747749, 748801, 748999 4.2.1.2 fumarate hydratase mitochondrion about 75% of total activity 5739 749006 4.2.1.2 fumarate hydratase mitochondrion Cos-1 cells transfected with fumarase constructs, human fumarase with either the native or cytochrome c oxidase subunit VIII mitochondrial targeting sequence is detected exclusively in mitochondria in more than 98% of the cells, while the remainder 1-2% of the cells shows varying amounts of nuclear labeling. When human fumarase is fused to the yeast mitochondrial targeting sequence, more than 50% of the cells show nuclear labeling 5739 677931 4.2.1.2 fumarate hydratase mitochondrion cytosolic fumarase and mitochondrial fumarase are identical products of the same nuclear gene 5739 33754 4.2.1.2 fumarate hydratase mitochondrion in thin sections from kidney, liver, heart, adrenal gland and anterior pituitary, strong and specific labeling due to fumarase antibody is only detected in mitochondria. In pancreatic acinar cells, in addition to mitochondria, highly significant labeling is also observed in the zymogen granules and endoplasmic reticulum 5739 680069 4.2.1.2 fumarate hydratase mitochondrion mitochondrial and cytosolic isoforms are derivatives of a single translation product and have identical amino termini 5739 652203 4.2.1.2 fumarate hydratase mitochondrion the cytosolic isoenzyme and the mitochondrial isoenzyme are identical over nearly all of their amino acid sequences but differ at their N-termini 5739 33753, 33758 4.2.1.2 fumarate hydratase mitochondrion the presequence of fumarase is exposed to the cytosol during import into mitochondria 5739 681379 4.2.1.2 fumarate hydratase mitochondrion translation product of the FUM1 gene, distribution can be affected by hsp70 molecular chaperones 5739 652477 4.2.1.2 fumarate hydratase mitochondrion under normal conditions 5739 706404 4.2.1.3 aconitate hydratase mitochondrion - 5739 -, 33784, 33786, 33797, 33799, 33807, 33808, 33810, 33813, 649551, 650165, 650198, 652036, 653825, 663589, 666289, 666296, 666327, 679969, 680182, 681116, 681864, 684713, 697220, 698948, 702863, 729779, 730672, 747087 4.2.1.3 aconitate hydratase mitochondrion 2 mitochondrial aconitases: M1 and M2 5739 653551 4.2.1.3 aconitate hydratase mitochondrion 24 h after intraperitoneal administration of endotoxin, there is a 28% reduction in mitochondrial respiration and a 24% reduction in aconitase activity. Functional activity of the electron transport chain is unaffected 5739 677832 4.2.1.3 aconitate hydratase mitochondrion 79% cytosolic enzyme and 21% mitochondrial enzyme for control animals, 83% cytosolic enzyme and 17% mitochondrial enzyme for starving animals 5739 650272 4.2.1.3 aconitate hydratase mitochondrion acclimation at 28°C results in strong decrease in enzyme mRNA and activity as well as in LON protease mRNA and activity 5739 679348 4.2.1.3 aconitate hydratase mitochondrion aconitase in yeast is a single translation product, which is dual targeted and distributed between the mitochondria and the cytosol by a unique mechanism involving reverse translocation, dual localization, detailed overview 5739 -, 715933 4.2.1.3 aconitate hydratase mitochondrion an age-related decrease in aconitase activity along with relatively subtle alterations in activities of other citric acid cycle enzymes may contribute to a decline in the overall efficiency of mitochondrial bioenergetics. The maximal activity of aconitase in mitochondria of 16-month-old (118 nmol aconitate/min/mg protein) and 24-month-old (108 nmol/min/mg protein) mice is consistently less than that from 6-month-old (147 aconitate/min/mg protein) mice 5739 748538 4.2.1.3 aconitate hydratase mitochondrion analysis of mitochondrial proteome, identification of two particular N-formylkynurenine modifications of enzyme 5739 681626 4.2.1.3 aconitate hydratase mitochondrion bL21-fused Aco2 protein resides in mitochondria as well as in the cytosol and the nucleus 5739 -, 747770 4.2.1.3 aconitate hydratase mitochondrion enzyme is a component of mitochondrial DNA nucleoids 5739 682550 4.2.1.3 aconitate hydratase mitochondrion localized both to the cytosol and mitochondria by a reverse translocation mechanism 5739 749367 4.2.1.3 aconitate hydratase mitochondrion mitochondrial aconitase activity represents up to 80% of the total aconitase activity in skin fibroblasts 5739 696992 4.2.1.3 aconitate hydratase mitochondrion mitochondrial isoform 5739 681552 4.2.1.3 aconitate hydratase mitochondrion mitochondrial isozyme 5739 716655 4.2.1.3 aconitate hydratase mitochondrion mitochondrial isozyme mAH 5739 696168 4.2.1.3 aconitate hydratase mitochondrion particulate-bound mitochondrial enzyme, the soluble mitochondrial enzyme is released from the mitochondria by freezing and thawing 5739 33784 4.2.1.3 aconitate hydratase mitochondrion significant decrease in activity with age, accompanied by relatively subtle alterations in activities of other citric acid cycle enzymes. Changes contribute to a decline in overall efficiency of mitochondrial bioenegetics with age 5739 681745 4.2.1.36 homoaconitate hydratase mitochondrion - 5739 5676 4.2.1.48 D-glutamate cyclase mitochondrion highly expressed in the mitochondrial matrix 5739 746927 4.2.1.48 D-glutamate cyclase mitochondrion matrix 5739 749348 4.2.1.59 3-hydroxyacyl-[acyl-carrier-protein] dehydratase mitochondrion - 5739 748986 4.2.1.74 medium-chain-enoyl-CoA hydratase mitochondrion - 5739 5866, 694522, 698680 4.2.1.74 medium-chain-enoyl-CoA hydratase mitochondrion inner membrane 5739 5869 4.2.1.9 dihydroxy-acid dehydratase mitochondrion - 5739 33960, 33969 4.2.1.9 dihydroxy-acid dehydratase mitochondrion matrix 5739 33965 4.2.1.9 dihydroxy-acid dehydratase mitochondrion predicted localisation 5739 730702 4.2.1.93 ATP-dependent NAD(P)H-hydrate dehydratase mitochondrion - 5739 -, 730041, 747020, 747751, 748172 4.2.1.93 ATP-dependent NAD(P)H-hydrate dehydratase mitochondrion import assays with purified pea (Pisum sativum) chloroplasts and mitochondria 5739 -, 728528 4.2.1.93 ATP-dependent NAD(P)H-hydrate dehydratase mitochondrion mCARKD 5739 747020 4.2.99.18 DNA-(apurinic or apyrimidinic site) lyase mitochondrion - 5739 648615, 648624, 681525, 682011, 682163, 692875, 747046 4.2.99.18 DNA-(apurinic or apyrimidinic site) lyase mitochondrion APE1 protein is also localized within mitochondria in different cell types, mitochondrial localization of APE1 is associated to a potential role in DNA repair of oxidized bases in the mitochondrial genome 5739 690474 4.2.99.18 DNA-(apurinic or apyrimidinic site) lyase mitochondrion mtAPE, N-terminal truncation product of APE1 5739 679468 4.2.99.18 DNA-(apurinic or apyrimidinic site) lyase mitochondrion the enzyme preferentially resides in the nucleus with conditional distribution in the mitochondria 5739 704663 4.2.99.B1 DNA 5'-deoxyribose phosphate lyase mitochondrion - 5739 692962, 694925 4.3.1.24 phenylalanine ammonia-lyase mitochondrion - 5739 34334 4.3.2.1 argininosuccinate lyase mitochondrion - 5739 34432 4.3.2.3 ureidoglycolate lyase mitochondrion - 5739 34477, 34478 4.4.1.1 cystathionine gamma-lyase mitochondrion - 5739 690376 4.4.1.13 cysteine-S-conjugate beta-lyase mitochondrion - 5739 -, 34547, 34551, 34555, 34557, 640043, 651545, 672202, 707092, 715070 4.4.1.13 cysteine-S-conjugate beta-lyase mitochondrion outer membrane 5739 34545 4.4.1.15 D-cysteine desulfhydrase mitochondrion - 5739 664881 4.4.1.17 Holocytochrome-c synthase mitochondrion - 5739 651895, 677440, 715034, 729264, 729740, 730058, 730322, 94056 4.4.1.17 Holocytochrome-c synthase mitochondrion detected by Western analysis 5739 653258 4.4.1.17 Holocytochrome-c synthase mitochondrion during apoptotic stimuli, enzyme translocates to outside the mitochondria, and binds to and suppresses the X-linked inhibitor of apoptosis protein XIAP, leading to activation of caspase-3. The N-terminus of the neuronal glutamate transporter excitatory amino-acid carrier 1 EAAC1 can bind to enzyme which interferes with the enzyme-XIAP association 5739 679489 4.4.1.17 Holocytochrome-c synthase mitochondrion integral membrane protein of inner membrane (facing cytoplasmic surface) 5739 94055 4.4.1.17 Holocytochrome-c synthase mitochondrion intermembrane space 5739 94057, 94058 4.4.1.17 Holocytochrome-c synthase mitochondrion mitoplast 5739 94055 4.4.1.17 Holocytochrome-c synthase mitochondrion novel, non-conservative import pathway: the enzyme precursor is translocated into mitochondria using the MOM19-GIP receptor complex in the outer membrane, independent of ATP-hydrolysis and electrochemical potential as external energy source 5739 94058 4.4.1.5 lactoylglutathione lyase mitochondrion - 5739 678043, 692049, 692050 4.4.1.5 lactoylglutathione lyase mitochondrion the enzyme is dual-targeted 5739 729176 4.4.1.9 L-3-cyanoalanine synthase mitochondrion - 5739 34719, 34724, 34727, 34728, 34736, 34737, 34738, 653464, 653511, 653555, 693422, 716516 4.4.1.9 L-3-cyanoalanine synthase mitochondrion phylogenetic analysis 5739 716554 4.4.1.9 L-3-cyanoalanine synthase mitochondrion the enzyme has a mitochondrial localization sequence 5739 730601 4.6.1.1 adenylate cyclase mitochondrion soluble isozyme 5739 693876 4.6.1.16 tRNA-intron lyase mitochondrion - 5739 698100, 727593 4.6.1.16 tRNA-intron lyase mitochondrion mitochondrial cytosolic surface 5739 646337 4.6.1.19 ribonuclease T2 mitochondrion - 5739 751115 4.98.1.1 protoporphyrin ferrochelatase mitochondrion - 5739 -, 34932, 34935, 34936, 34937, 34938, 34939, 34944, 34947, 34948, 34950, 34953, 34955, 34959, 34961, 34965, 34967, 34969, 34974, 665534, 678251, 702862, 705158, 706101, 716139, 730588, 747213, 763125 4.98.1.1 protoporphyrin ferrochelatase mitochondrion active mature protein, 62 amino acid leader sequence is sufficient for targeting of a leader sequence-YFP fusion protein to mitochondria, but does not allow cleavage from YFP 5739 681946 4.98.1.1 protoporphyrin ferrochelatase mitochondrion inner membrane 5739 34933, 34943, 34957, 34962, 34973 4.98.1.1 protoporphyrin ferrochelatase mitochondrion inner mitochondrial membrane 5739 714523, 715203 4.98.1.1 protoporphyrin ferrochelatase mitochondrion little activity, latent enzyme 5739 649728 4.98.1.1 protoporphyrin ferrochelatase mitochondrion membrane 5739 34946, 34949, 34956 4.98.1.1 protoporphyrin ferrochelatase mitochondrion the mitochondrial ferrochelatase activity in a FC1 overexpressor line increases 5fold in comparison with wild-type mitochondria 5739 748928 5.1.2.6 isocitrate epimerase mitochondrion - 5739 2224 5.1.99.1 methylmalonyl-CoA epimerase mitochondrion - 5739 661687, 676032, 747542 5.1.99.4 alpha-methylacyl-CoA racemase mitochondrion - 5739 680093, 681308, 681814, 682220, 703387, 704836, 705416, 727322, 747334 5.1.99.4 alpha-methylacyl-CoA racemase mitochondrion co-distributed exclusively with mitochondrial marker enzymes 5739 2447 5.1.99.4 alpha-methylacyl-CoA racemase mitochondrion only 10-30% of the activity is found in mitochondria 5739 2447 5.1.99.6 NAD(P)H-hydrate epimerase mitochondrion - 5739 728528 5.1.99.6 NAD(P)H-hydrate epimerase mitochondrion mainly 5739 726917 5.2.1.2 maleylacetoacetate isomerase mitochondrion - 5739 746642 5.2.1.8 peptidylprolyl isomerase mitochondrion - 5739 2514 5.2.1.8 peptidylprolyl isomerase mitochondrion cyclosporin A-sensitive activity is largely localized to the mitochondrial matrix. Rapamycin-sensitive activity is associated with the mitochondrial membranes 5739 651842 5.2.1.8 peptidylprolyl isomerase mitochondrion matrix 5739 2537, 660983 5.2.1.8 peptidylprolyl isomerase mitochondrion mitochondrial form not detected 5739 2526 5.3.3.1 steroid DELTA-isomerase mitochondrion - 5739 2900 5.3.3.2 isopentenyl-diphosphate DELTA-isomerase mitochondrion - 5739 2934, 727623, 728493 5.3.3.2 isopentenyl-diphosphate DELTA-isomerase mitochondrion enhanced green fluorescent protein fusions are found mainly in the mitochondrion 5739 694638 5.3.3.21 DELTA3,5-DELTA2,4-dienoyl-CoA isomerase mitochondrion - 5739 744206, 744290, 745257, 745262, 745267, 746519 5.3.3.7 aconitate DELTA-isomerase mitochondrion low activity 5739 2996 5.3.3.8 DELTA3-DELTA2-enoyl-CoA isomerase mitochondrion - 5739 2997, 2998, 3000, 3005, 3008, 3009, 3013, 3014, 3015, 3016, 3021, 3023, 3025, 649676, 652365, 662630, 663340, 678385, 691485, 706005, 728221, 747742 5.3.3.8 DELTA3-DELTA2-enoyl-CoA isomerase mitochondrion ECI and MECI 5739 652357 5.3.3.8 DELTA3-DELTA2-enoyl-CoA isomerase mitochondrion enzyme is synthesized with a N-terminal mitochondrial targeting sequence which is cleaved during mitochondrial import 5739 3026 5.3.3.8 DELTA3-DELTA2-enoyl-CoA isomerase mitochondrion in addition to the peroxisomal trifunctional enzyme and the mitochondrial enzyme which shows a preference for short-chain substrates, a separate isomerase with a preference for C10-C12 substrates is observed 5739 3018 5.3.3.8 DELTA3-DELTA2-enoyl-CoA isomerase mitochondrion main intracellular site 5739 3024 5.3.3.8 DELTA3-DELTA2-enoyl-CoA isomerase mitochondrion short chain DELTA3,DELTA2-enoyl-CoA isomerase 5739 3022 5.3.4.1 protein disulfide-isomerase mitochondrion presence of isoforms P5 and Erp57. P5 localizes to the inner side of the inner membrane 5739 692913 5.3.99.2 Prostaglandin-D synthase mitochondrion enzyme from basophilc leukemia cell line RBL-1 5739 3125 5.4.3.7 leucine 2,3-aminomutase mitochondrion activity is 100fold lower than in cytosol of liver 5739 3415 5.4.99.2 methylmalonyl-CoA mutase mitochondrion - 5739 -, 677443, 701956, 705936, 715560, 726874, 727036, 728310, 746727 5.4.99.2 methylmalonyl-CoA mutase mitochondrion enzyme hMCM preferably locates in mitochondria, with small amounts of protein in the cytoplasm 5739 747209 5.4.99.42 tRNA pseudouridine31 synthase mitochondrion - 5739 712349 5.4.99.44 mitochondrial tRNA pseudouridine27/28 synthase mitochondrion efficient and rapid targeting of Pus2p in yeast mitochondria 5739 -, 682837 5.4.99.B22 multisite-specific tRNA pseudouridine synthase mitochondrion - 5739 748225 5.6.1.3 plus-end-directed kinesin ATPase mitochondrion - 5739 210552 5.6.1.3 plus-end-directed kinesin ATPase mitochondrion kinesin light chain KLC3 can associate with mitochobdria 5739 668365 5.6.1.3 plus-end-directed kinesin ATPase mitochondrion the motor is adapted to special requirements of mitochondrial shaping. A high local motor accumulation may allow microtubule interaction and local expansion of the mitochondrium 5739 669173 5.6.1.7 chaperonin ATPase mitochondrion - 5739 289221, 667512, 668723, 687714 5.6.1.7 chaperonin ATPase mitochondrion CPN10 colocalizes with the GroES homologue CPN60.2 5739 670103 5.6.1.7 chaperonin ATPase mitochondrion GroES homologue CPN60.2 5739 670103 5.6.2.1 DNA topoisomerase mitochondrion - 5739 3813, 690902 5.6.2.1 DNA topoisomerase mitochondrion isozymes Top1mt, Top3alpha and a truncated form of Top2beta 5739 728579 5.6.2.1 DNA topoisomerase mitochondrion liver 5739 3813 5.6.2.1 DNA topoisomerase mitochondrion membrane-associated 5739 3803 5.6.2.1 DNA topoisomerase mitochondrion oocytes 5739 3813 5.6.2.2 DNA topoisomerase (ATP-hydrolysing) mitochondrion - 5739 3811, 3883, 3899, 694241, 694962 5.6.2.2 DNA topoisomerase (ATP-hydrolysing) mitochondrion no topoisomerase specific for mitochondria 5739 3808 5.6.2.2 DNA topoisomerase (ATP-hydrolysing) mitochondrion the mitochondrial enzyme is a truncated version of DNA topoisomerase IIbeta 5739 651160 5.6.2.3 DNA 5'-3' helicase mitochondrion - 5739 719883, 720012, 720591, 756521, 757836, 757850, 763236, 763249, 763430, 763526 5.6.2.3 DNA 5'-3' helicase mitochondrion the Arabidopsis Twinkle homologue enzyme is dual targeted to mitochondria and chloroplasts 5739 733586 5.6.2.3 DNA 5'-3' helicase mitochondrion Twinkle homologue (Twm1) in Dictyostelium discoideum is encoded by the nuclear twm1 gene and targeted to mitochondria 5739 756304 5.6.2.4 DNA 3'-5' helicase mitochondrion mitochondrial localization of Hmi1p is essential for its role in mtDNA metabolism 5739 695273 6.1.1.1 tyrosine-tRNA ligase mitochondrion - 5739 662832, 694895, 695113, 705174, 714246 6.1.1.1 tyrosine-tRNA ligase mitochondrion human mitochondrial and cytosolic enzymes are encoded by two different sets of genes 5739 658121 6.1.1.1 tyrosine-tRNA ligase mitochondrion the mt-TyrRS is strictly mitochondrial, mitochondrial idiosyncrasies 5739 677165 6.1.1.10 methionine-tRNA ligase mitochondrion - 5739 662940, 704223 6.1.1.10 methionine-tRNA ligase mitochondrion a single gene of chloroplast origin codes for mitochondrial and chloroplastic isozyme, possessing a targeting signal sequence with dual function 5739 653613 6.1.1.10 methionine-tRNA ligase mitochondrion hmMetRS 5739 701744 6.1.1.10 methionine-tRNA ligase mitochondrion mitochondrial isozyme 5739 651401 6.1.1.10 methionine-tRNA ligase mitochondrion mitochondrial isozyme contains no zinc-finger motif and no insertion domain in the nucleotide-binding fold 5739 652862 6.1.1.10 methionine-tRNA ligase mitochondrion mtMetRS 5739 661156 6.1.1.10 methionine-tRNA ligase mitochondrion the isozyme from mitochondria is identical with the one from chloroplasts 5739 653613 6.1.1.11 serine-tRNA ligase mitochondrion - 5739 652204, 653486, 660582, 661593, 92 6.1.1.11 serine-tRNA ligase mitochondrion dual localization is established by the virtue of an ambiguous targeting peptid at the very N-terminus 5739 694659 6.1.1.11 serine-tRNA ligase mitochondrion mitochondrial isozyme 5739 652010 6.1.1.11 serine-tRNA ligase mitochondrion mitochondrial isozyme SerZMm 5739 649451 6.1.1.11 serine-tRNA ligase mitochondrion mtSerRS localizes exclusively in the mitochondria 5739 660869 6.1.1.12 aspartate-tRNA ligase mitochondrion - 5739 658121, 676127, 693741, 702481, 704488, 714091, 726911, 728404, 744436 6.1.1.14 glycine-tRNA ligase mitochondrion - 5739 -, 652038, 671098, 706875, 728297, 728398, 746265, 92 6.1.1.14 glycine-tRNA ligase mitochondrion gene contains a mitochondrial targeting sequence 5739 651399 6.1.1.14 glycine-tRNA ligase mitochondrion gene product of GRS1 has enzymic activity both in cytoplasm and mitochondria 5739 662193 6.1.1.14 glycine-tRNA ligase mitochondrion isozyme GlyRS2, enzyme is imported from the cytosol, nucleus-encoded 5739 652111 6.1.1.14 glycine-tRNA ligase mitochondrion isozymes GlyRS1 and GlyRS2, enzymes are imported from the cytosol, nucleus-encoded, isozyme GlyRS1 is inactive in the mitochondria 5739 652111 6.1.1.14 glycine-tRNA ligase mitochondrion isozymes GlyRS1, enzyme is imported from the cytosol, nucleus-encoded, isozyme GlyRS1 is inactive in the mitochondria 5739 652111 6.1.1.15 proline-tRNA ligase mitochondrion - 5739 170, 92 6.1.1.15 proline-tRNA ligase mitochondrion isozyme ProRS-Org 5739 746082 6.1.1.16 cysteine-tRNA ligase mitochondrion - 5739 745531, 745862, 92 6.1.1.17 glutamate-tRNA ligase mitochondrion - 5739 702746, 706514 6.1.1.18 glutamine-tRNA ligase mitochondrion - 5739 659311, 92 6.1.1.18 glutamine-tRNA ligase mitochondrion localization of cytoplasmic GlnRS in mitochondrial Gln-tRNA synthesis. Saccharomyces cerevisiae imports the cytosolic pathway for Gln-tRNA synthesis into the mitochondrion 5739 661830 6.1.1.19 arginine-tRNA ligase mitochondrion - 5739 671204, 727625 6.1.1.2 tryptophan-tRNA ligase mitochondrion - 5739 651999 6.1.1.2 tryptophan-tRNA ligase mitochondrion isozyme TrpRS2 contains a mitochondrial targeting signal, two genes exist due to the need for a mitochondrial synthetase with extended substrate specificity to achieve aminoacylation of the imported thiolated and edited tRNATrp 5739 676863 6.1.1.2 tryptophan-tRNA ligase mitochondrion WARS2 5739 705840 6.1.1.20 phenylalanine-tRNA ligase mitochondrion - 5739 334, 338, 340, 341, 343, 669399, 671135, 706561, 715937, 727095, 727288, 746395, 746396 6.1.1.20 phenylalanine-tRNA ligase mitochondrion mitochondrial isozyme 5739 652794 6.1.1.20 phenylalanine-tRNA ligase mitochondrion mitochondrial PheRS is nuclear-encoded 5739 695155 6.1.1.20 phenylalanine-tRNA ligase mitochondrion mtPheRS 5739 706561 6.1.1.21 histidine-tRNA ligase mitochondrion - 5739 365, 716387, 716785, 744663, 92 6.1.1.21 histidine-tRNA ligase mitochondrion targeting sequence on the N-terminal extension 5739 651244 6.1.1.22 asparagine-tRNA ligase mitochondrion - 5739 745056, 746169, 92 6.1.1.23 aspartate-tRNAAsn ligase mitochondrion - 5739 693741 6.1.1.24 glutamate-tRNAGln ligase mitochondrion - 5739 728399 6.1.1.3 threonine-tRNA ligase mitochondrion - 5739 -, 395, 705818, 727916, 728681, 743906, 745918, 92 6.1.1.4 leucine-tRNA ligase mitochondrion - 5739 427, 443, 653766, 661190, 662619, 672244, 674619, 674686, 675436, 714322, 727364, 744788, 744849, 744968 6.1.1.4 leucine-tRNA ligase mitochondrion mitochondrial isozyme 5739 650328 6.1.1.5 isoleucine-tRNA ligase mitochondrion - 5739 745057 6.1.1.5 isoleucine-tRNA ligase mitochondrion large isoform 5739 -, 728395 6.1.1.6 lysine-tRNA ligase mitochondrion - 5739 -, 673690, 675713, 693146, 744283, 744901, 745444 6.1.1.6 lysine-tRNA ligase mitochondrion cytosolic tRNAsLys tRK1 is partially targeted into mitochondria. Binding of tRK1 to the precursor of mitochondrial lysyl-tRNA synthetase preMsk1p, IIb class aminoacyl-tRNA synthetase, is a prerequisite of import. The hinge region of preMsk1p is essential to provide the import. The N-terminal domain of preMsk1p with the adjacent hinge region not only interacts with aminoacylated tRK1, but is also able to direct tRK1 import even in absence of the C-terminal domain. Enolase 2 participates as a putative RNA chaperone, suggesting that it may induce the import-active conformation in the tRNA 5739 705591 6.1.1.6 lysine-tRNA ligase mitochondrion mitochondrial isozyme 5739 652053 6.1.1.6 lysine-tRNA ligase mitochondrion of HIV-1-producing 293FT cells 5739 662738 6.1.1.7 alanine-tRNA ligase mitochondrion - 5739 -, 653639, 662300, 662826, 726653, 744969, 745765, 92 6.1.1.7 alanine-tRNA ligase mitochondrion very poor mitochondrial targeting activity of the ALA1 leader peptide, mapping of the mitochondrial targeting signal, overview 5739 676064 6.1.1.9 valine-tRNA ligase mitochondrion - 5739 535, 538, 673963, 674764, 694436, 714322, 726908, 746112 6.1.1.9 valine-tRNA ligase mitochondrion Schizosaccharomyces pombe contains two distinct nuclear ValRS genes, one encoding the mitochondrial form and the other its cytosolic counterpart. The mitochondrial form exhibits significant divergence from other yeast ValRS sequences. Gene is active and essential for the survival of the yeast. The mitochondrial form lacks the characteristic appended domain 5739 705623 6.2.1.1 acetate-CoA ligase mitochondrion - 5739 562, 567, 674377, 716267 6.2.1.1 acetate-CoA ligase mitochondrion AceCS2 5739 676801 6.2.1.1 acetate-CoA ligase mitochondrion aerobic isoenzyme 5739 582 6.2.1.1 acetate-CoA ligase mitochondrion matrix 5739 676800 6.2.1.1 acetate-CoA ligase mitochondrion matrix, AceCS2 5739 652101 6.2.1.10 carboxylic acid-CoA ligase (GDP-forming) mitochondrion - 5739 563, 584, 585, 586 6.2.1.10 carboxylic acid-CoA ligase (GDP-forming) mitochondrion exclusively associated with the inner membrane-matrix 5739 587 6.2.1.15 arachidonate-CoA ligase mitochondrion - 5739 715838, 716687 6.2.1.16 acetoacetate-CoA ligase mitochondrion - 5739 651 6.2.1.17 propionate-CoA ligase mitochondrion - 5739 654, 656, 657, 659, 660 6.2.1.17 propionate-CoA ligase mitochondrion mitochondrial matrix 5739 745242 6.2.1.2 medium-chain acyl-CoA ligase mitochondrion - 5739 562, 649709, 649783, 649791, 650831, 651834, 672, 675, 676959, 677, 678, 679, 681 6.2.1.2 medium-chain acyl-CoA ligase mitochondrion in vivo the enzyme is linked to a mitochondrial membrane by a bond cleavable by disulfide bond reducing agents 5739 680 6.2.1.2 medium-chain acyl-CoA ligase mitochondrion matrix 5739 670 6.2.1.24 phytanate-CoA ligase mitochondrion - 5739 702, 704, 705 6.2.1.3 long-chain-fatty-acid-CoA ligase mitochondrion - 5739 661519, 662072, 662463, 671606, 674830, 714313, 714678, 715838, 728840, 738, 742, 746, 752, 756, 758, 762, 763, 764 6.2.1.3 long-chain-fatty-acid-CoA ligase mitochondrion ACSl5 is the only isoform of long chain acyl-CoA synthetase partly located on mitochondria. Ad-ACSL-5, long chain acyl-CoA synthetase 5 overexpressed in rat hepatoma McRrdle-RG7777 cells mediated by adenovirus colocalizes to both mitochondria and endoplasmic reticulum 5739 662463 6.2.1.3 long-chain-fatty-acid-CoA ligase mitochondrion enzyme form acyl-CoA synthetase I 5739 759 6.2.1.3 long-chain-fatty-acid-CoA ligase mitochondrion isoform ACSL5 5739 746549 6.2.1.3 long-chain-fatty-acid-CoA ligase mitochondrion mitochondrial membrane, ACS5. ACS1 is not detected in mitochondria 5739 652139 6.2.1.4 succinate-CoA ligase (GDP-forming) mitochondrion - 5739 650949, 662256, 672980, 687185, 693488, 714342, 728078, 745498, 783, 787 6.2.1.5 succinate-CoA ligase (ADP-forming) mitochondrion - 5739 650949, 660655, 673835, 687185, 693488, 702332, 714342, 727919, 728078, 745498, 746481, 797, 803, 818, 819 6.2.1.76 malonate-CoA ligase mitochondrion - 5739 763858, 764430, 765034, 765038, 765555, 765574 6.2.1.76 malonate-CoA ligase mitochondrion human ACSF3 is located exclusively in mitochondria 5739 765038 6.3.1.2 glutamine synthetase mitochondrion - 5739 37504 6.3.1.2 glutamine synthetase mitochondrion liver enzyme is targeted to mitochondria. Two different glutamine synthetase transcripts are generated by tissue-specific alternative splicing. The liver transcript contains an alternative exon that is not present in the neural one. This exon leads to acquisition of an upstream in-frame start codon and formation of a mitochondrial targeting signal 5739 661765 6.3.1.2 glutamine synthetase mitochondrion the enzyme is spread throughout the cytoplasm and inside the mitochondrial lumen in all life cycle forms 5739 -, 743570 6.3.1.2 glutamine synthetase mitochondrion the GLN2 gene product functions in both leaf mitochondria and chloroplasts to faciliate ammonium recovery during photorespiration 5739 663056 6.3.1.2 glutamine synthetase mitochondrion the N-terminus of glutamine synthetase, which constitutes a weak mitochondrial targeting signal, is sufficient to direct a chimeric protein to the mitochondria in hepatocytes and to the cytoplasm in astrocytes. The mitochondrial membrane potential in hepatocytes is more negative than in astrocytes 5739 715711 6.3.1.20 lipoate-protein ligase mitochondrion - 5739 -, 662098, 706425, 737026, 745964, 746406 6.3.1.20 lipoate-protein ligase mitochondrion in intraerythrocytic parasites, enzyme catalyzes incorporation of lipoate to mitochondrial proteins 5739 676079 6.3.2.12 dihydrofolate synthase mitochondrion - 5739 1012, 1020, 1026 6.3.2.17 tetrahydrofolate synthase mitochondrion - 5739 1092, 649432, 650764, 651989, 653665, 672921, 675734, 728522, 744775, 745311 6.3.2.17 tetrahydrofolate synthase mitochondrion 20% of the mycelial FPGS is mitochondrial 5739 1082 6.3.2.17 tetrahydrofolate synthase mitochondrion a single gene encodes both, mitochondrial and cytosolic isoforms of the enzyme 5739 649260 6.3.2.17 tetrahydrofolate synthase mitochondrion leaf 5739 1090 6.3.2.17 tetrahydrofolate synthase mitochondrion mFGPS is at least in part strongly associated with the inner mitochondrial membrane 5739 661265 6.3.2.17 tetrahydrofolate synthase mitochondrion MTHFD1L enzyme is present and functional in mitochondria from normal embryonic tissues and embryonic fibroblast cell lines 5739 712483 6.3.2.2 glutamate-cysteine ligase mitochondrion - 5739 694060 6.3.2.3 glutathione synthase mitochondrion isozyme GSHS1 5739 648906 6.3.2.3 glutathione synthase mitochondrion of nodule 5739 648906 6.3.3.1 phosphoribosylformylglycinamidine cyclo-ligase mitochondrion - 5739 653485, 653535 6.3.3.2 5-formyltetrahydrofolate cyclo-ligase mitochondrion - 5739 1329, 662384 6.3.3.2 5-formyltetrahydrofolate cyclo-ligase mitochondrion exclusively 5739 652326 6.3.3.2 5-formyltetrahydrofolate cyclo-ligase mitochondrion matrix 5739 1330 6.3.3.2 5-formyltetrahydrofolate cyclo-ligase mitochondrion small amounts 5739 1328 6.3.3.3 dethiobiotin synthase mitochondrion BIO3 protein contains an N-terminal sequence that is predicted to target the protein to mitochondria 5739 694716 6.3.4.10 biotin-[propionyl-CoA-carboxylase (ATP-hydrolysing)] ligase mitochondrion - 5739 1389, 1396, 701936 6.3.4.15 biotin-[biotin carboxyl-carrier protein] ligase mitochondrion - 5739 701936 6.3.4.16 carbamoyl-phosphate synthase (ammonia) mitochondrion - 5739 -, 1450, 1452, 1454, 1465, 1466, 1467, 1468, 1469, 1476, 652202, 652660, 672441, 673178, 675160, 675859, 691725, 693935, 702101, 702142, 702953, 703149, 705524, 726867, 727610, 728044, 745931 6.3.4.3 formate-tetrahydrofolate ligase mitochondrion - 5739 652471, 701402 6.3.4.3 formate-tetrahydrofolate ligase mitochondrion mitochondrial C1-THF synthase behaves as a peripheral membrane protein, tightly associated with the matrix side of the mitochondrial inner membrane 5739 690632 6.3.4.3 formate-tetrahydrofolate ligase mitochondrion mitochondrial isozyme 5739 660817 6.3.4.3 formate-tetrahydrofolate ligase mitochondrion mitochondrial isozyme, contains an N-terminal mitochondrial targeting sequence 5739 660866 6.3.4.3 formate-tetrahydrofolate ligase mitochondrion mitochondrial monofunctional isozyme 5739 662447 6.3.4.3 formate-tetrahydrofolate ligase mitochondrion MTHFD1L enzyme is present in mitochondria from normal embryonic tissues and embryonic fibroblast cell lines, and embryonic mitochondria possess the ability to synthesize formate from glycine. Greater than 75% of 1-C units entering the cytoplasmic methyl cycle are mitochondrially derived 5739 712483 6.3.4.3 formate-tetrahydrofolate ligase mitochondrion MTHFD1L is a mitochondrial monofunctional enzyme with 10-CHO-THF synthase activity 5739 765141, 765861 6.3.5.3 phosphoribosylformylglycinamidine synthase mitochondrion - 5739 694724 6.3.5.4 asparagine synthase (glutamine-hydrolysing) mitochondrion - 5739 -, 675860, 745294 6.3.5.5 carbamoyl-phosphate synthase (glutamine-hydrolysing) mitochondrion - 5739 -, 1730, 697908, 743989, 744975 6.3.5.5 carbamoyl-phosphate synthase (glutamine-hydrolysing) mitochondrion CPS III 5739 652660 6.3.5.7 glutaminyl-tRNA synthase (glutamine-hydrolysing) mitochondrion - 5739 706514 6.4.1.1 pyruvate carboxylase mitochondrion - 5739 1758, 1774, 1775, 1784, 1788, 1796, 1799, 649546, 649744, 650952, 652312, 662759, 692005, 693143, 714040 6.4.1.2 acetyl-CoA carboxylase mitochondrion - 5739 664564, 675028, 675087, 744144, 744275 6.4.1.2 acetyl-CoA carboxylase mitochondrion 3fold higher activity than in etioplasts 5739 653543 6.4.1.2 acetyl-CoA carboxylase mitochondrion Hfa1p is a mitochondrion-specific enzyme and contains a canonical mitochondrial targeting sequence with a matrix protease cleavage site 5739 -, 665515 6.4.1.2 acetyl-CoA carboxylase mitochondrion long enzyme form 5739 664193 6.4.1.2 acetyl-CoA carboxylase mitochondrion mitochondrion-specific isozyme ACC2 5739 664193 6.4.1.2 acetyl-CoA carboxylase mitochondrion mitochondrion-specific isozyme Hfa1p 5739 664193 6.4.1.3 propionyl-CoA carboxylase mitochondrion - 5739 1870, 1876, 651406, 651459, 652691, 653242, 653246, 716328, 745501, 745766 6.4.1.4 methylcrotonoyl-CoA carboxylase mitochondrion - 5739 -, 1870, 1883, 1885, 1893, 1895, 652968, 653128, 653498, 653530, 662287, 662746, 662747, 713750 6.5.1.1 DNA ligase (ATP) mitochondrion - 5739 1925, 661615, 663223, 713775, 714201, 716334, 716335, 716669 6.5.1.3 RNA ligase (ATP) mitochondrion - 5739 -, 1996, 1997, 650982, 651858, 653217, 653869, 653889 7.1.1.1 proton-translocating NAD(P)+ transhydrogenase mitochondrion - 5739 742240, 764039, 764052, 764194, 764699, 764928, 765163, 765255, 765259, 765744 7.1.1.2 NADH:ubiquinone reductase (H+-translocating) mitochondrion - 5739 -, 391082, 392676, 392677, 392679, 392684, 392687, 392692, 392699, 392700, 392701, 392703, 392707, 392709, 392717, 392718, 392721, 657845, 658135, 659106, 659387, 659946, 660055, 671370, 671827, 671993, 672013, 672126, 672129, 672176, 672291, 672347, 672348, 672597, 672974, 673448, 673615, 673620, 673672, 673940, 674467, 674495, 674521, 675654, 690388, 690427, 690713, 690886, 691037, 691039, 691049, 691058, 691773, 692181, 692231, 692447, 692643, 692992, 693532, 693720, 693745, 693773, 693815, 694192, 694352, 694393, 694936, 695063, 695189, 696270, 696288, 696510, 696745, 698312, 699152, 707505, 714281, 714303, 714993, 726118, 726174, 741606, 742879, 743263, 743265, 743268 7.1.1.2 NADH:ubiquinone reductase (H+-translocating) mitochondrion constitutively high content 5739 392690 7.1.1.2 NADH:ubiquinone reductase (H+-translocating) mitochondrion DE2 faces the intermembrane space 5739 714300 7.1.1.2 NADH:ubiquinone reductase (H+-translocating) mitochondrion inner membrane 5739 392683, 392696 7.1.1.2 NADH:ubiquinone reductase (H+-translocating) mitochondrion loosly bound to inner mitochondrial membrane 5739 392696 7.1.1.2 NADH:ubiquinone reductase (H+-translocating) mitochondrion membrane 5739 392685, 392686, 392690, 392702, 392708 7.1.1.2 NADH:ubiquinone reductase (H+-translocating) mitochondrion NADH:ubiquinone oxidoreductase (complex I) from bovine heart mitochondria is a membrane-bound enzyme that contains 45 different subunits 5739 696312 7.1.1.2 NADH:ubiquinone reductase (H+-translocating) mitochondrion submitochondrial fragments 5739 725002 7.1.1.2 NADH:ubiquinone reductase (H+-translocating) mitochondrion the enzyme oxidizes NADH in the mitochondrial matrix and reduces ubiquinone in the inner mitochondrial membrane 5739 671882 7.1.1.2 NADH:ubiquinone reductase (H+-translocating) mitochondrion the level of respiratory complexes I is lower in mitochondria from cells grown at pH 6.0 than in mitochondria from cells grown at pH 3.7 5739 739328 7.1.1.2 NADH:ubiquinone reductase (H+-translocating) mitochondrion the mitochondrial multienzyme-complex is of dual origin. 6 of the at least 22 subunits with MW of 70000 Da, 48000 Da, 37000 Da, 25000 Da, 22000 Da and 18000 Da are synthesized in mitochondria. 11 subunits are synthesized in the cytoplasm 5739 392688 7.1.1.8 quinol-cytochrome-c reductase mitochondrion - 5739 -, 395300, 396001, 439942, 439944, 439945, 439946, 439948, 439949, 439950, 439951, 439952, 439953, 439955, 439956, 439958, 439961, 439964, 439966, 439967, 439969, 439970, 439972, 439973, 439975, 439977, 439978, 439979, 439982, 439983, 439984, 439986, 439987, 439988, 439989, 439990, 439991, 439992, 439993, 439995, 439997, 440003, 440006, 440008, 440009, 440011, 440012, 440014, 440015, 440018, 440020, 440023, 440024, 440025, 440026, 440027, 440028, 440030, 440035, 657683, 658009, 658161, 658165, 658194, 658674, 659443, 672379, 674163, 674433, 674484, 674721, 685905, 686076, 696100, 700002, 724452, 725396, 742055, 742224, 742760, 743061, 750065 7.1.1.8 quinol-cytochrome-c reductase mitochondrion matrix space of mitochondria and intermembrane space 5739 440004 7.1.1.8 quinol-cytochrome-c reductase mitochondrion mitochondrial membrane, transmembranous subunit QPc-9.5 kDa, more mass on the matrix side of the membrane 5739 439994, 440016 7.1.1.8 quinol-cytochrome-c reductase mitochondrion subsarcolemmal 5739 659673 7.1.1.8 quinol-cytochrome-c reductase mitochondrion the level of respiratory complexes III is lower in mitochondria from cells grown at pH 6.0 than in mitochondria from cells grown at pH 3.7 5739 739327 7.1.1.9 cytochrome-c oxidase mitochondrion - 5739 -, 657776, 658173, 659507, 660201, 674427, 674791, 675538, 688882, 689159, 690357, 690457, 691023, 691038, 691048, 691050, 691059, 691404, 692222, 692575, 692992, 693006, 693451, 693722, 693787, 693985, 694175, 694263, 694360, 694377, 694379, 694394, 694535, 694592, 694694, 694959, 694987, 695076, 695140, 695205, 695551, 697203, 698272, 698934, 699003, 699277, 699664, 700077, 700420, 700992, 711279, 711289, 712032, 712326, 712814, 713249, 713547, 724436, 741541, 742002, 742031, 742243, 742590, 742816, 743020, 743264, 743634, 753813, 755271, 755489 7.1.1.9 cytochrome-c oxidase mitochondrion in intact mitochondria, CO binds to two heme proteins, CytcO and flavohemoglobin yHb. yHb resides in the intermembrane space. In vesicles of the inner mitochondrial membranes the only CO-binding protein is CytcO 5739 752804 7.1.1.9 cytochrome-c oxidase mitochondrion intermembrane space 5739 -, 692985 7.1.1.9 cytochrome-c oxidase mitochondrion mitochondrion-endoded subunit COXII does not localize exclusively to mitochondria, but is also present in cytosol. COXIV RNA-binding activity, present in the mitochondrial fraction, decreases during development with inverse correlation with accumulation of COXIV precursor in the cytoplasm 5739 660014 7.1.2.2 H+-transporting two-sector ATPase mitochondrion - 5739 -, 210229, 210232, 210233, 210237, 210239, 210241, 210244, 210245, 210249, 210250, 210251, 210254, 210257, 210262, 210264, 672313, 673318, 674212, 676871, 676946, 685315, 698308, 698676, 700972, 713098, 719502, 734656, 747488, 747824, 748800, 749153 7.1.2.2 H+-transporting two-sector ATPase mitochondrion in vitro the subunit 8 of F1F0-ATPase can be inserted post-translationally across the inverted mitochondrial membrane vesicles with the correct trans-cis topology depending on the mitochondrial matrix fraction but independently of ATP, membrane potential, or protein components exposed to the matrix space 5739 656007 7.1.2.2 H+-transporting two-sector ATPase mitochondrion inner membrane 5739 210232 7.1.2.2 H+-transporting two-sector ATPase mitochondrion mitochondrial FOF1 ATP synthase 5739 712325 7.1.2.2 H+-transporting two-sector ATPase mitochondrion mitochondrial translation of the Saccharomyces cerevisiae Atp6p subunit of F1-F0 ATP synthase is regulated by the F1 ATPase 5739 719502 7.1.2.2 H+-transporting two-sector ATPase mitochondrion progressive declining enzyme activity at supra-optimal salinities 5739 673361 7.1.3.1 H+-exporting diphosphatase mitochondrion 23% of activity 5739 654467 7.2.1.1 NADH:ubiquinone reductase (Na+-transporting) mitochondrion - 5739 671351 7.2.2.14 P-type Mg2+ transporter mitochondrion - 5739 -, 685621, 701311 7.2.2.14 P-type Mg2+ transporter mitochondrion seasonal variations in Mg2+-ATPase activity of rat heart mitochondria 5739 700652 7.2.2.3 P-type Na+ transporter mitochondrion - 5739 654200 7.4.2.10 ABC-type glutathione transporter mitochondrion - 5739 752257 7.4.2.3 mitochondrial protein-transporting ATPase mitochondrion - 5739 -, 672943, 673628, 673805, 674473, 674522, 675002, 675964, 676134, 676773, 698800, 698945, 712482, 734275, 734764, 751175 7.4.2.3 mitochondrial protein-transporting ATPase mitochondrion in vitro import of the fusion proteins depends on Gly repeat lengths and folded mature domains. Presequence and Gly repeat function as an mtHsp70 binding site and mtHsp70 avoidance segment, respectively 5739 698809 7.4.2.3 mitochondrial protein-transporting ATPase mitochondrion inner membrane 5739 656343, 657154 7.4.2.3 mitochondrial protein-transporting ATPase mitochondrion inner mitochondrial membrane 5739 210436, 210437, 210438, 210439, 210440, 210441 7.4.2.3 mitochondrial protein-transporting ATPase mitochondrion to study the biochemical functions the components of the mtHsp70 machinery, Hsp70, J-protein, GrpE, and Hep are reconstituted 5739 712438 7.4.2.5 bacterial ABC-type protein transporter mitochondrion - 5739 656145 7.6.2.4 ABC-type fatty-acyl-CoA transporter mitochondrion - 5739 667893 7.6.2.5 ABC-type heme transporter mitochondrion - 5739 289162, 289165, 289167