EC Number |
Recommended Name |
Localization |
GeneOntology No. |
Reference |
---|
2.5.1.10 | (2E,6E)-farnesyl diphosphate synthase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
737749 |
2.5.1.B14 | (2E,6E)-farnesyl-diphosphate all-trans-nonaprenyl diphosphate synthase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
704891, 759800 |
2.5.1.B14 | (2E,6E)-farnesyl-diphosphate all-trans-nonaprenyl diphosphate synthase |
mitochondrion |
isoform SPS1 |
GO:0005739 AmiGO QuickGO |
759800 |
2.5.1.B14 | (2E,6E)-farnesyl-diphosphate all-trans-nonaprenyl diphosphate synthase |
mitochondrion |
isoform SPS1 shows a putative mitochondrial signal peptide sequence |
GO:0005739 AmiGO QuickGO |
704891 |
2.5.1.B14 | (2E,6E)-farnesyl-diphosphate all-trans-nonaprenyl diphosphate synthase |
mitochondrion |
mitchondrial isozyme OsSPS1 |
GO:0005739 AmiGO QuickGO |
739259 |
2.5.1.92 | (2Z,6Z)-farnesyl diphosphate synthase |
mitochondrion |
probably |
GO:0005739 AmiGO QuickGO |
-, 723434 |
2.4.1.92 | (N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
489574 |
2.6.1.40 | (R)-3-amino-2-methylpropionate-pyruvate transaminase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
737947 |
1.1.1.75 | (R)-aminopropanol dehydrogenase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
287300, 287301, 287307 |
1.1.3.15 | (S)-2-hydroxy-acid oxidase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
389700, 389701, 741109 |
1.1.3.15 | (S)-2-hydroxy-acid oxidase |
mitochondrion |
preparation of heavy and light mitochondria, 2 distinct molecular forms |
GO:0005739 AmiGO QuickGO |
389693 |
1.1.5.13 | (S)-2-hydroxyglutarate dehydrogenase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
755031 |
2.6.1.22 | (S)-3-amino-2-methylpropionate transaminase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
636964 |
2.5.1.74 | 1,4-dihydroxy-2-naphthoate polyprenyltransferase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
737563 |
2.3.1.51 | 1-acylglycerol-3-phosphate O-acyltransferase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
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 |
GO:0005739 AmiGO QuickGO |
-, 486375 |
2.3.1.23 | 1-acylglycerophosphocholine O-acyltransferase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
486621, 486629, 657864 |
2.3.1.23 | 1-acylglycerophosphocholine O-acyltransferase |
mitochondrion |
probably associated with the outer membrane |
GO:0005739 AmiGO QuickGO |
486647 |
1.1.1.B40 | 11beta-hydroxysteroid dehydrogenase (NAD+) |
mitochondrion |
isozyme 11beta-HSD2 |
GO:0005739 AmiGO QuickGO |
667203 |
2.1.1.B125 | 16S rRNA (guanine1145-2'-O)-methyltransferase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
757147 |
2.1.1.B124 | 16S rRNA (guanine1370-2'-O)-methyltransferase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
757147, 757634 |
2.1.1.B123 | 16S rRNA (uridine1369-2'-O)-methyltransferase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
757633 |
1.14.14.32 | 17alpha-hydroxyprogesterone deacetylase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
677795 |
1.1.1.62 | 17beta-estradiol 17-dehydrogenase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
760700, 761037 |
1.1.1.62 | 17beta-estradiol 17-dehydrogenase |
mitochondrion |
isozyme 17beta-HSD10 |
GO:0005739 AmiGO QuickGO |
675983 |
1.1.1.62 | 17beta-estradiol 17-dehydrogenase |
mitochondrion |
isozyme AKR1B15.1 |
GO:0005739 AmiGO QuickGO |
740743 |
3.1.4.37 | 2',3'-cyclic-nucleotide 3'-phosphodiesterase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
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 |
GO:0005739 AmiGO QuickGO |
-, 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. |
GO:0005739 AmiGO QuickGO |
670268 |
3.1.4.37 | 2',3'-cyclic-nucleotide 3'-phosphodiesterase |
mitochondrion |
isoform II, intermembrane space |
GO:0005739 AmiGO QuickGO |
730475 |
3.1.4.37 | 2',3'-cyclic-nucleotide 3'-phosphodiesterase |
mitochondrion |
the enzyme is associated with complexes I-V in mitochondria |
GO:0005739 AmiGO QuickGO |
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 |
GO:0005739 AmiGO QuickGO |
730760 |
2.7.7.84 | 2'-5' oligoadenylate synthase |
mitochondrion |
OAS1 p46 isoform |
GO:0005739 AmiGO QuickGO |
762506 |
2.7.1.76 | 2'-deoxyadenosine kinase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
739204 |
2.4.99.20 | 2'-phospho-ADP-ribosyl cyclase/2'-phospho-cyclic-ADP-ribose transferase |
mitochondrion |
0.2 nM/min*mg |
GO:0005739 AmiGO QuickGO |
724210 |
2.7.1.160 | 2'-phosphotransferase |
mitochondrion |
2'-phosphotransferase shows a tendency to associate with mitochondria |
GO:0005739 AmiGO QuickGO |
685412 |
1.3.1.34 | 2,4-dienoyl-CoA reductase [(2E)-enoyl-CoA-producing] |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
390710, 652365, 657265, 759709 |
1.3.1.124 | 2,4-dienoyl-CoA reductase [(3E)-enoyl-CoA-producing] |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 390707, 390709, 390712, 390714, 390715, 390716, 390718, 390719, 390722, 654376, 656286, 685405, 690046, 700872, 758746, 759137, 759825, 760087, 760176 |
2.7.6.3 | 2-amino-4-hydroxy-6-hydroxymethyldihydropteridine diphosphokinase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
674784 |
2.7.6.3 | 2-amino-4-hydroxy-6-hydroxymethyldihydropteridine diphosphokinase |
mitochondrion |
mitHPPK/DHPS |
GO:0005739 AmiGO QuickGO |
674784 |
2.6.1.39 | 2-aminoadipate transaminase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 636620, 636622, 636623, 639979, 703133 |
2.6.1.39 | 2-aminoadipate transaminase |
mitochondrion |
glutamate-alpha-ketoadipate transaminase I |
GO:0005739 AmiGO QuickGO |
639974 |
2.6.1.39 | 2-aminoadipate transaminase |
mitochondrion |
isoenzyme AadAT-II |
GO:0005739 AmiGO QuickGO |
639983 |
2.2.1.5 | 2-hydroxy-3-oxoadipate synthase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
395925, 395926, 395930, 395933, 395935 |
2.2.1.5 | 2-hydroxy-3-oxoadipate synthase |
mitochondrion |
exclusively |
GO:0005739 AmiGO QuickGO |
395924 |
2.2.1.5 | 2-hydroxy-3-oxoadipate synthase |
mitochondrion |
part of alpha-ketoglutarate dehydrogenase complex |
GO:0005739 AmiGO QuickGO |
349030, 395924, 395936 |
2.1.1.373 | 2-hydroxy-4-(methylsulfanyl)butanoate S-methyltransferase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 759840 |
2.3.3.13 | 2-isopropylmalate synthase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 736032 |
2.3.3.13 | 2-isopropylmalate synthase |
mitochondrion |
alpha-isopropylmalate synthase Ia, MW 68000, is imported into the mitochondrial matrix |
GO:0005739 AmiGO QuickGO |
636532 |
2.3.3.13 | 2-isopropylmalate synthase |
mitochondrion |
large enzyme form, matrix |
GO:0005739 AmiGO QuickGO |
-, 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 |
GO:0005739 AmiGO QuickGO |
636507 |
3.1.3.106 | 2-lysophosphatidate phosphatase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
754054 |
2.1.1.201 | 2-methoxy-6-polyprenyl-1,4-benzoquinol methylase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
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 |
GO:0005739 AmiGO QuickGO |
735704 |
2.3.3.5 | 2-methylcitrate synthase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 5344, 735795 |
2.3.3.5 | 2-methylcitrate synthase |
mitochondrion |
enzyme possesses a mitochondrial signal import sequence |
GO:0005739 AmiGO QuickGO |
488089, 661700 |
4.1.1.71 | 2-oxoglutarate decarboxylase |
mitochondrion |
exclusively |
GO:0005739 AmiGO QuickGO |
648364 |
4.1.1.71 | 2-oxoglutarate decarboxylase |
mitochondrion |
synaptic and nonsynaptic |
GO:0005739 AmiGO QuickGO |
349037 |
1.2.1.105 | 2-oxoglutarate dehydrogenase system |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
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 |
GO:0005739 AmiGO QuickGO |
673870 |
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 |
GO:0005739 AmiGO QuickGO |
485140 |
1.1.1.150 | 21-hydroxysteroid dehydrogenase (NAD+) |
mitochondrion |
isoform RDH13 |
GO:0005739 AmiGO QuickGO |
688677 |
2.1.1.168 | 21S rRNA (uridine2791-2'-O)-methyltransferase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
697605 |
1.1.1.51 | 3(or 17)beta-hydroxysteroid dehydrogenase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
287077, 677079 |
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 |
GO:0005739 AmiGO QuickGO |
674407 |
1.14.99.60 | 3-demethoxyubiquinol 3-hydroxylase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
746696, 746956, 748142 |
1.14.99.60 | 3-demethoxyubiquinol 3-hydroxylase |
mitochondrion |
mitochondrial inner membrane protein |
GO:0005739 AmiGO QuickGO |
748155 |
2.1.1.64 | 3-demethylubiquinol 3-O-methyltransferase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
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 |
GO:0005739 AmiGO QuickGO |
485140 |
2.7.7.38 | 3-deoxy-manno-octulosonate cytidylyltransferase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
723388 |
1.1.1.178 | 3-hydroxy-2-methylbutyryl-CoA dehydrogenase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
673530, 711851 |
1.1.1.35 | 3-hydroxyacyl-CoA dehydrogenase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 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 |
GO:0005739 AmiGO QuickGO |
286603 |
4.2.1.59 | 3-hydroxyacyl-[acyl-carrier-protein] dehydratase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
748986 |
1.14.13.24 | 3-hydroxybenzoate 6-monooxygenase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 728255 |
1.1.1.30 | 3-hydroxybutyrate dehydrogenase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
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 |
GO:0005739 AmiGO QuickGO |
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 |
GO:0005739 AmiGO QuickGO |
685409 |
1.1.1.30 | 3-hydroxybutyrate dehydrogenase |
mitochondrion |
located on the matrix face of the inner membrane |
GO:0005739 AmiGO QuickGO |
286512, 286513, 286533, 286538 |
1.1.1.31 | 3-hydroxyisobutyrate dehydrogenase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
688515, 722472 |
3.1.2.4 | 3-hydroxyisobutyryl-CoA hydrolase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
653127, 94558 |
1.1.1.59 | 3-hydroxypropionate dehydrogenase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
738640 |
2.8.1.2 | 3-mercaptopyruvate sulfurtransferase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
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 |
GO:0005739 AmiGO QuickGO |
-, 645554 |
2.8.1.2 | 3-mercaptopyruvate sulfurtransferase |
mitochondrion |
isoforms TUM1-Iso2 |
GO:0005739 AmiGO QuickGO |
738634 |
2.8.1.2 | 3-mercaptopyruvate sulfurtransferase |
mitochondrion |
partly |
GO:0005739 AmiGO QuickGO |
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 |
GO:0005739 AmiGO QuickGO |
761523 |
1.2.4.4 | 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
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 |
GO:0005739 AmiGO QuickGO |
94884 |
1.3.1.22 | 3-oxo-5alpha-steroid 4-dehydrogenase (NADP+) |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
396212 |
2.8.3.5 | 3-oxoacid CoA-transferase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
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 |
GO:0005739 AmiGO QuickGO |
645972 |
2.8.3.5 | 3-oxoacid CoA-transferase |
mitochondrion |
rat heart mitochondria at 4, 13, 19, and 24 months of age |
GO:0005739 AmiGO QuickGO |
690880 |
2.8.3.5 | 3-oxoacid CoA-transferase |
mitochondrion |
the enzyme is a mitochondrial homodimer |
GO:0005739 AmiGO QuickGO |
694193 |
1.1.1.100 | 3-oxoacyl-[acyl-carrier-protein] reductase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
704721, 740558 |
2.8.3.6 | 3-oxoadipate CoA-transferase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 756853 |
2.3.1.174 | 3-oxoadipyl-CoA thiolase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 756853 |
4.2.1.107 | 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA hydratase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
658438 |
1.17.99.3 | 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
659114 |
1.1.1.277 | 3beta-hydroxy-5beta-steroid dehydrogenase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 389563 |