EC Number |
Recommended Name |
Localization |
GeneOntology No. |
Reference |
---|
6.1.1.11 | serine-tRNA ligase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
652204, 653486, 660582, 661593, 92 |
6.1.1.18 | glutamine-tRNA ligase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
659311, 92 |
6.1.1.15 | proline-tRNA ligase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
170, 92 |
6.1.1.20 | phenylalanine-tRNA ligase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
334, 338, 340, 341, 343, 669399, 671135, 706561, 715937, 727095, 727288, 746395, 746396 |
6.1.1.21 | histidine-tRNA ligase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
365, 716387, 716785, 744663, 92 |
6.2.1.1 | acetate-CoA ligase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
562, 567, 674377, 716267 |
6.2.1.10 | carboxylic acid-CoA ligase (GDP-forming) |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
563, 584, 585, 586 |
6.2.1.1 | acetate-CoA ligase |
mitochondrion |
aerobic isoenzyme |
GO:0005739 AmiGO QuickGO |
582 |
6.2.1.10 | carboxylic acid-CoA ligase (GDP-forming) |
mitochondrion |
exclusively associated with the inner membrane-matrix |
GO:0005739 AmiGO QuickGO |
587 |
6.2.1.16 | acetoacetate-CoA ligase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
651 |
6.2.1.17 | propionate-CoA ligase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
654, 656, 657, 659, 660 |
6.2.1.2 | medium-chain acyl-CoA ligase |
mitochondrion |
matrix |
GO:0005739 AmiGO QuickGO |
670 |
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 |
GO:0005739 AmiGO QuickGO |
680 |
6.2.1.24 | phytanate-CoA ligase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
702, 704, 705 |
6.2.1.3 | long-chain-fatty-acid-CoA ligase |
mitochondrion |
enzyme form acyl-CoA synthetase I |
GO:0005739 AmiGO QuickGO |
759 |
6.2.1.4 | succinate-CoA ligase (GDP-forming) |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
650949, 662256, 672980, 687185, 693488, 714342, 728078, 745498, 783, 787 |
6.2.1.5 | succinate-CoA ligase (ADP-forming) |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
650949, 660655, 673835, 687185, 693488, 702332, 714342, 727919, 728078, 745498, 746481, 797, 803, 818, 819 |
6.3.2.12 | dihydrofolate synthase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
1012, 1020, 1026 |
6.3.2.17 | tetrahydrofolate synthase |
mitochondrion |
20% of the mycelial FPGS is mitochondrial |
GO:0005739 AmiGO QuickGO |
1082 |
6.3.2.17 | tetrahydrofolate synthase |
mitochondrion |
leaf |
GO:0005739 AmiGO QuickGO |
1090 |
6.3.2.17 | tetrahydrofolate synthase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
1092, 649432, 650764, 651989, 653665, 672921, 675734, 728522, 744775, 745311 |
6.3.3.2 | 5-formyltetrahydrofolate cyclo-ligase |
mitochondrion |
small amounts |
GO:0005739 AmiGO QuickGO |
1328 |
6.3.3.2 | 5-formyltetrahydrofolate cyclo-ligase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
1329, 662384 |
6.3.3.2 | 5-formyltetrahydrofolate cyclo-ligase |
mitochondrion |
matrix |
GO:0005739 AmiGO QuickGO |
1330 |
6.3.4.10 | biotin-[propionyl-CoA-carboxylase (ATP-hydrolysing)] ligase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
1389, 1396, 701936 |
6.3.4.16 | carbamoyl-phosphate synthase (ammonia) |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 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 |
1.2.1.38 | N-acetyl-gamma-glutamyl-phosphate reductase |
mitochondrion |
soluble matrix enzyme |
GO:0005739 AmiGO QuickGO |
1730 |
6.4.1.1 | pyruvate carboxylase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
1758, 1774, 1775, 1784, 1788, 1796, 1799, 649546, 649744, 650952, 652312, 662759, 692005, 693143, 714040 |
6.4.1.3 | propionyl-CoA carboxylase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
1870, 1876, 651406, 651459, 652691, 653242, 653246, 716328, 745501, 745766 |
6.5.1.3 | RNA ligase (ATP) |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 1996, 1997, 650982, 651858, 653217, 653869, 653889 |
5.1.2.6 | isocitrate epimerase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
2224 |
5.1.99.4 | alpha-methylacyl-CoA racemase |
mitochondrion |
co-distributed exclusively with mitochondrial marker enzymes |
GO:0005739 AmiGO QuickGO |
2447 |
5.1.99.4 | alpha-methylacyl-CoA racemase |
mitochondrion |
only 10-30% of the activity is found in mitochondria |
GO:0005739 AmiGO QuickGO |
2447 |
5.2.1.8 | peptidylprolyl isomerase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
2514 |
5.2.1.8 | peptidylprolyl isomerase |
mitochondrion |
mitochondrial form not detected |
GO:0005739 AmiGO QuickGO |
2526 |
5.2.1.8 | peptidylprolyl isomerase |
mitochondrion |
matrix |
GO:0005739 AmiGO QuickGO |
2537, 660983 |
5.3.3.1 | steroid DELTA-isomerase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
2900 |
5.3.3.7 | aconitate DELTA-isomerase |
mitochondrion |
low activity |
GO:0005739 AmiGO QuickGO |
2996 |
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 |
GO:0005739 AmiGO QuickGO |
3018 |
5.3.3.8 | DELTA3-DELTA2-enoyl-CoA isomerase |
mitochondrion |
short chain DELTA3,DELTA2-enoyl-CoA isomerase |
GO:0005739 AmiGO QuickGO |
3022 |
5.3.3.8 | DELTA3-DELTA2-enoyl-CoA isomerase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
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 |
main intracellular site |
GO:0005739 AmiGO QuickGO |
3024 |
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 |
GO:0005739 AmiGO QuickGO |
3026 |
5.3.99.2 | Prostaglandin-D synthase |
mitochondrion |
enzyme from basophilc leukemia cell line RBL-1 |
GO:0005739 AmiGO QuickGO |
3125 |
5.4.3.7 | leucine 2,3-aminomutase |
mitochondrion |
activity is 100fold lower than in cytosol of liver |
GO:0005739 AmiGO QuickGO |
3415 |
5.6.2.1 | DNA topoisomerase |
mitochondrion |
membrane-associated |
GO:0005739 AmiGO QuickGO |
3803 |
5.6.2.2 | DNA topoisomerase (ATP-hydrolysing) |
mitochondrion |
no topoisomerase specific for mitochondria |
GO:0005739 AmiGO QuickGO |
3808 |
5.6.2.2 | DNA topoisomerase (ATP-hydrolysing) |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
3811, 3883, 3899, 694241, 694962 |
5.6.2.1 | DNA topoisomerase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
3813, 690902 |
5.6.2.1 | DNA topoisomerase |
mitochondrion |
liver |
GO:0005739 AmiGO QuickGO |
3813 |
5.6.2.1 | DNA topoisomerase |
mitochondrion |
oocytes |
GO:0005739 AmiGO QuickGO |
3813 |
4.1.1.19 | arginine decarboxylase |
mitochondrion |
highly if not exclusively associated with mitochondrial membrane |
GO:0005739 AmiGO QuickGO |
4097 |
4.1.1.29 | sulfinoalanine decarboxylase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
4238 |
4.1.1.112 | oxaloacetate decarboxylase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
4270, 756006 |
4.1.1.112 | oxaloacetate decarboxylase |
mitochondrion |
differs from cytoplasmic enzyme |
GO:0005739 AmiGO QuickGO |
4271 |
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 |
GO:0005739 AmiGO QuickGO |
4345 |
4.1.1.32 | phosphoenolpyruvate carboxykinase (GTP) |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
4344, 4349, 4350, 4353, 4359, 4367, 667608, 678205, 679083, 702944, 704540, 727993, 748770 |
4.1.1.40 | hydroxypyruvate decarboxylase |
mitochondrion |
75% of the enzyme activity |
GO:0005739 AmiGO QuickGO |
4543 |
4.1.1.42 | carnitine decarboxylase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
4555 |
4.1.1.9 | malonyl-CoA decarboxylase |
mitochondrion |
intramitochondrial distribution |
GO:0005739 AmiGO QuickGO |
4843 |
4.1.1.9 | malonyl-CoA decarboxylase |
mitochondrion |
matrix space |
GO:0005739 AmiGO QuickGO |
4845 |
4.1.1.9 | malonyl-CoA decarboxylase |
mitochondrion |
tightly bound |
GO:0005739 AmiGO QuickGO |
4846 |
4.1.1.9 | malonyl-CoA decarboxylase |
mitochondrion |
a small portion is present in mitochondria |
GO:0005739 AmiGO QuickGO |
4854 |
4.1.1.9 | malonyl-CoA decarboxylase |
mitochondrion |
matrix |
GO:0005739 AmiGO QuickGO |
4854 |
4.1.1.9 | malonyl-CoA decarboxylase |
mitochondrion |
enzyme from liver |
GO:0005739 AmiGO QuickGO |
4858 |
4.1.3.16 | 4-Hydroxy-2-oxoglutarate aldolase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
5237, 713711 |
4.1.3.16 | 4-Hydroxy-2-oxoglutarate aldolase |
mitochondrion |
89% of total activity |
GO:0005739 AmiGO QuickGO |
5253 |
4.1.3.30 | Methylisocitrate lyase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 5344, 714846 |
4.1.3.4 | hydroxymethylglutaryl-CoA lyase |
mitochondrion |
87% of activity |
GO:0005739 AmiGO QuickGO |
5360 |
4.1.3.4 | hydroxymethylglutaryl-CoA lyase |
mitochondrion |
exclusively |
GO:0005739 AmiGO QuickGO |
5364 |
4.1.3.4 | hydroxymethylglutaryl-CoA lyase |
mitochondrion |
matrix |
GO:0005739 AmiGO QuickGO |
5362, 5364, 5372 |
4.2.1.36 | homoaconitate hydratase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
5676 |
4.2.1.74 | medium-chain-enoyl-CoA hydratase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
5866, 694522, 698680 |
4.2.1.74 | medium-chain-enoyl-CoA hydratase |
mitochondrion |
inner membrane |
GO:0005739 AmiGO QuickGO |
5869 |
1.1.3.37 | D-arabinono-1,4-lactone oxidase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
10663 |
3.4.21.89 | Signal peptidase I |
mitochondrion |
inner membrane |
GO:0005739 AmiGO QuickGO |
29813 |
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 |
GO:0005739 AmiGO QuickGO |
31388 |
3.4.24.64 | mitochondrial processing peptidase |
mitochondrion |
beta-MPP: membrane-associated |
GO:0005739 AmiGO QuickGO |
31402, 31406 |
3.4.24.64 | mitochondrial processing peptidase |
mitochondrion |
integral part of bc1-complex of respiratory chain |
GO:0005739 AmiGO QuickGO |
31404 |
3.4.24.64 | mitochondrial processing peptidase |
mitochondrion |
alpha-MPP: soluble |
GO:0005739 AmiGO QuickGO |
31406 |
2.3.1.16 | acetyl-CoA C-acyltransferase |
mitochondrion |
binding to inner membrane |
GO:0005739 AmiGO QuickGO |
33713 |
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 |
GO:0005739 AmiGO QuickGO |
33753, 33758 |
4.2.1.2 | fumarate hydratase |
mitochondrion |
cytosolic fumarase and mitochondrial fumarase are identical products of the same nuclear gene |
GO:0005739 AmiGO QuickGO |
33754 |
4.2.1.3 | aconitate hydratase |
mitochondrion |
particulate-bound mitochondrial enzyme, the soluble mitochondrial enzyme is released from the mitochondria by freezing and thawing |
GO:0005739 AmiGO QuickGO |
33784 |
4.2.1.9 | dihydroxy-acid dehydratase |
mitochondrion |
matrix |
GO:0005739 AmiGO QuickGO |
33965 |
1.1.1.86 | ketol-acid reductoisomerase (NADP+) |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
33969, 684636 |
2.2.1.6 | acetolactate synthase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 33969, 395883, 395890, 395896, 718641, 734734 |
4.2.1.9 | dihydroxy-acid dehydratase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
33960, 33969 |
4.3.1.24 | phenylalanine ammonia-lyase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
34334 |
4.3.2.1 | argininosuccinate lyase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
34432 |
4.3.2.3 | ureidoglycolate lyase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
34477, 34478 |
4.4.1.13 | cysteine-S-conjugate beta-lyase |
mitochondrion |
outer membrane |
GO:0005739 AmiGO QuickGO |
34545 |
4.4.1.9 | L-3-cyanoalanine synthase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
34719, 34724, 34727, 34728, 34736, 34737, 34738, 653464, 653511, 653555, 693422, 716516 |
4.98.1.1 | protoporphyrin ferrochelatase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 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 |
membrane |
GO:0005739 AmiGO QuickGO |
34946, 34949, 34956 |
4.98.1.1 | protoporphyrin ferrochelatase |
mitochondrion |
inner membrane |
GO:0005739 AmiGO QuickGO |
34933, 34943, 34957, 34962, 34973 |
3.4.11.3 | cystinyl aminopeptidase |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
35902, 35918 |
3.4.24.16 | neurolysin |
mitochondrion |
EP24.16m is the mitochondrial enzyme form |
GO:0005739 AmiGO QuickGO |
37061 |
3.4.24.16 | neurolysin |
mitochondrion |
- |
GO:0005739 AmiGO QuickGO |
-, 37063, 37077, 668828, 754221, 754607, 754898 |
3.4.24.16 | neurolysin |
mitochondrion |
intermembrane space |
GO:0005739 AmiGO QuickGO |
37085 |