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| | DARS2 | Aspartyl-tRNA synthetase 2, mitochondrial. (645 aa) |
| | WARS1 | Tryptophanyl-tRNA synthetase; Belongs to the class-I aminoacyl-tRNA synthetase family. (470 aa) |
| | SUCLG2 | Succinate--CoA ligase [GDP-forming] subunit beta, mitochondrial; GTP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit. (432 aa) |
| | RNF165 | Ring finger protein 165. (347 aa) |
| | GLUL | Glutamate-ammonia ligase; Belongs to the glutamine synthetase family. (353 aa) |
| | TTLL2 | Tubulin tyrosine ligase like 2. (519 aa) |
| | H0Y1G0_OTOGA | Uncharacterized protein. (211 aa) |
| | LGSN | Lengsin, lens protein with glutamine synthetase domain; Belongs to the glutamine synthetase family. (572 aa) |
| | H0Y160_OTOGA | Uncharacterized protein. (770 aa) |
| | SAE1 | SUMO1 activating enzyme subunit 1. (348 aa) |
| | FPGS | Folylpolyglutamate synthase; Catalyzes conversion of folates to polyglutamate derivatives allowing concentration of folate compounds in the cell and the intracellular retention of these cofactors, which are important substrates for most of the folate-dependent enzymes that are involved in one-carbon transfer reactions involved in purine, pyrimidine and amino acid synthesis; Belongs to the folylpolyglutamate synthase family. (587 aa) |
| | MDM2 | E3 ubiquitin-protein ligase Mdm2; Belongs to the MDM2/MDM4 family. (493 aa) |
| | H0XWM1_OTOGA | AMP-binding domain-containing protein. (656 aa) |
| | PFAS | Phosphoribosylformylglycinamidine synthase. (1338 aa) |
| | H0XVM3_OTOGA | Protein SDA1; Required for 60S pre-ribosomal subunits export to the cytoplasm; Belongs to the SDA1 family. (668 aa) |
| | PARS2 | Prolyl-tRNA synthetase 2, mitochondrial. (475 aa) |
| | H0XUE2_OTOGA | Uncharacterized protein. (212 aa) |
| | ASS1 | Argininosuccinate synthase 1. (414 aa) |
| | PPCS | Phosphopantothenoylcysteine synthetase. (311 aa) |
| | MOCS3 | Adenylyltransferase and sulfurtransferase MOCS3; Plays a central role in 2-thiolation of mcm(5)S(2)U at tRNA wobble positions of cytosolic tRNA(Lys), tRNA(Glu) and tRNA(Gln). Also essential during biosynthesis of the molybdenum cofactor. Acts by mediating the C-terminal thiocarboxylation of sulfur carriers URM1 and MOCS2A. Its N-terminus first activates URM1 and MOCS2A as acyl- adenylates (-COAMP), then the persulfide sulfur on the catalytic cysteine is transferred to URM1 and MOCS2A to form thiocarboxylation (- COSH) of their C-terminus. The reaction probably involves hydrogen sulfide [...] (460 aa) |
| | ADSS2 | Adenylosuccinate synthetase isozyme 2; Plays an important role in the de novo pathway and in the salvage pathway of purine nucleotide biosynthesis. Catalyzes the first commited step in the biosynthesis of AMP from IMP. (462 aa) |
| | MARS2 | Methionyl-tRNA synthetase 2, mitochondrial; Belongs to the class-I aminoacyl-tRNA synthetase family. (593 aa) |
| | ACSL5 | Acyl-CoA synthetase long chain family member 5. (682 aa) |
| | ATP5F1E | ATP synthase F1 subunit epsilon. (51 aa) |
| | TPGS1 | Tubulin polyglutamylase complex subunit 1. (292 aa) |
| | TTLL10 | Tubulin tyrosine ligase like 10. (613 aa) |
| | ATP5F1D | ATP synthase F1 subunit delta. (168 aa) |
| | LOC100958823 | Diphthine--ammonia ligase; Amidase that catalyzes the last step of diphthamide biosynthesis using ammonium and ATP. (267 aa) |
| | ATP6V0C | V-type proton ATPase proteolipid subunit; Proton-conducting pore forming subunit of the membrane integral V0 complex of vacuolar ATPase. V-ATPase is responsible for acidifying a variety of intracellular compartments in eukaryotic cells. (155 aa) |
| | SLC27A5 | Solute carrier family 27 member 5. (689 aa) |
| | MROH6 | Maestro heat like repeat family member 6. (705 aa) |
| | UCHL1 | Ubiquitin carboxyl-terminal hydrolase. (252 aa) |
| | ACSM4 | Acyl-CoA synthetase medium chain family member 4. (581 aa) |
| | H0XL26_OTOGA | ATP-synt_ab_N domain-containing protein. (141 aa) |
| | LIG4 | DNA ligase. (915 aa) |
| | H0XJE4_OTOGA | Uncharacterized protein. (279 aa) |
| | RARS2 | Arginyl-tRNA synthetase 2, mitochondrial; Belongs to the class-I aminoacyl-tRNA synthetase family. (578 aa) |
| | UBE3A | Ubiquitin-protein ligase E3A; E3 ubiquitin-protein ligase which accepts ubiquitin from an E2 ubiquitin-conjugating enzyme in the form of a thioester and transfers it to its substrates. (851 aa) |
| | PCCA | Propionyl-CoA carboxylase subunit alpha. (728 aa) |
| | CAD | Carbamoyl-phosphate synthetase 2, aspartate transcarbamylase, and dihydroorotase. (2223 aa) |
| | ITCH | E3 ubiquitin-protein ligase. (853 aa) |
| | ACSM1 | Acyl-CoA synthetase medium chain family member 1. (574 aa) |
| | ACSL3 | Acyl-CoA synthetase long chain family member 3. (720 aa) |
| | NARS1 | Asparaginyl-tRNA synthetase. (560 aa) |
| | ATG7 | Ubiquitin-like modifier-activating enzyme ATG7; E1-like activating enzyme involved in the 2 ubiquitin-like systems required for cytoplasm to vacuole transport (Cvt) and autophagy. Activates ATG12 for its conjugation with ATG5 as well as the ATG8 family proteins for their conjugation with phosphatidylethanolamine. Both systems are needed for the ATG8 association to Cvt vesicles and autophagosomes membranes. Required for autophagic death induced by caspase-8 inhibition. Required for mitophagy which contributes to regulate mitochondrial quantity and quality by eliminating the mitochondria [...] (652 aa) |
| | ATP5PO | Mitochondrial ATP synthase, O subunit (Predicted). (213 aa) |
| | GART | Trifunctional purine biosynthetic protein adenosine-3; In the C-terminal section; belongs to the GART family. In the central section; belongs to the AIR synthase family. (1010 aa) |
| | LIPT2 | Putative lipoyltransferase 2, mitochondrial; Catalyzes the transfer of endogenously produced octanoic acid from octanoyl-acyl-carrier-protein onto the lipoyl domains of lipoate- dependent enzymes. Lipoyl-ACP can also act as a substrate although octanoyl-ACP is likely to be the physiological substrate. Belongs to the LipB family. (231 aa) |
| | UBA1 | Ubiquitin like modifier activating enzyme 1; Belongs to the ubiquitin-activating E1 family. (1059 aa) |
| | IARS1 | Isoleucyl-tRNA synthetase; Belongs to the class-I aminoacyl-tRNA synthetase family. (1263 aa) |
| | ADSS1 | Adenylosuccinate synthetase isozyme 1; Component of the purine nucleotide cycle (PNC), which interconverts IMP and AMP to regulate the nucleotide levels in various tissues, and which contributes to glycolysis and ammoniagenesis. Catalyzes the first commited step in the biosynthesis of AMP from IMP. (463 aa) |
| | H0XD62_OTOGA | CTP synthase; Catalyzes the ATP-dependent amination of UTP to CTP with either L-glutamine or ammonia as the source of nitrogen. (591 aa) |
| | ATP5F1C | ATP synthase subunit gamma. (298 aa) |
| | UBE3B | Ubiquitin protein ligase E3B. (1068 aa) |
| | TTLL11 | Tubulin tyrosine ligase like 11. (723 aa) |
| | NAPRT | Nicotinate phosphoribosyltransferase; Catalyzes the first step in the biosynthesis of NAD from nicotinic acid, the ATP-dependent synthesis of beta-nicotinate D- ribonucleotide from nicotinate and 5-phospho-D-ribose 1-phosphate. Belongs to the NAPRTase family. (537 aa) |
| | CCDC30 | Coiled-coil domain containing 30. (773 aa) |
| | ACSL4 | Acyl-CoA synthetase long chain family member 4. (711 aa) |
| | ACSS3 | Acyl-CoA synthetase short chain family member 3. (689 aa) |
| | NAE1 | NEDD8-activating enzyme E1 regulatory subunit; Regulatory subunit of the dimeric UBA3-NAE1 E1 enzyme. E1 activates NEDD8 by first adenylating its C-terminal glycine residue with ATP, thereafter linking this residue to the side chain of the catalytic cysteine, yielding a NEDD8-UBA3 thioester and free AMP. E1 finally transfers NEDD8 to the catalytic cysteine of UBE2M. Belongs to the ubiquitin-activating E1 family. ULA1 subfamily. (534 aa) |
| | GCLM | Glutamate-cysteine ligase modifier subunit. (274 aa) |
| | BTRC | Beta-transducin repeat containing E3 ubiquitin protein ligase. (526 aa) |
| | UBA6 | Ubiquitin like modifier activating enzyme 6. (1052 aa) |
| | TARS3 | Threonyl-tRNA synthetase like 2. (805 aa) |
| | GATB | Glutamyl-tRNA(Gln) amidotransferase subunit B, mitochondrial; Allows the formation of correctly charged Gln-tRNA(Gln) through the transamidation of misacylated Glu-tRNA(Gln) in the mitochondria. The reaction takes place in the presence of glutamine and ATP through an activated gamma-phospho-Glu-tRNA(Gln). Belongs to the GatB/GatE family. GatB subfamily. (556 aa) |
| | ACACA | Acetyl-CoA carboxylase alpha. (2348 aa) |
| | SARS1 | Seryl-tRNA synthetase. (514 aa) |
| | GARS1 | Glycyl-tRNA synthetase. (739 aa) |
| | TTLL8 | Tubulin tyrosine ligase like 8. (583 aa) |
| | C17orf80 | Chromosome 17 open reading frame 80. (611 aa) |
| | DIP2A | Disco interacting protein 2 homolog A. (1578 aa) |
| | ASNSD1 | Glutamine amidotransferase type-2 domain-containing protein. (631 aa) |
| | QRSL1 | Glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial; Allows the formation of correctly charged Gln-tRNA(Gln) through the transamidation of misacylated Glu-tRNA(Gln) in the mitochondria. The reaction takes place in the presence of glutamine and ATP through an activated gamma-phospho-Glu-tRNA(Gln). Belongs to the amidase family. GatA subfamily. (527 aa) |
| | TTLL9 | Tubulin tyrosine ligase like 9. (461 aa) |
| | LOC100953148 | Uncharacterized protein. (578 aa) |
| | FARSA | Phenylalanyl-tRNA synthetase subunit alpha. (508 aa) |
| | NARS2 | Asparaginyl-tRNA synthetase 2, mitochondrial. (477 aa) |
| | TTLL6 | Tubulin tyrosine ligase like 6. (622 aa) |
| | MTHFS | 5-formyltetrahydrofolate cyclo-ligase; Belongs to the 5-formyltetrahydrofolate cyclo-ligase family. (203 aa) |
| | SDAD1 | Protein SDA1; Required for 60S pre-ribosomal subunits export to the cytoplasm; Belongs to the SDA1 family. (688 aa) |
| | AARS1 | Alanyl-tRNA synthetase; Catalyzes the attachment of alanine to tRNA(Ala) in a two- step reaction: alanine is first activated by ATP to form Ala-AMP and then transferred to the acceptor end of tRNA(Ala). Also edits incorrectly charged tRNA(Ala) via its editing domain. Belongs to the class-II aminoacyl-tRNA synthetase family. (968 aa) |
| | ACSM3 | Acyl-CoA synthetase medium chain family member 3. (586 aa) |
| | YARS2 | Tyrosine--tRNA ligase. (469 aa) |
| | RIMKLB | Ribosomal modification protein rimK like family member B. (386 aa) |
| | IARS2 | Isoleucyl-tRNA synthetase 2, mitochondrial; Belongs to the class-I aminoacyl-tRNA synthetase family. (1003 aa) |
| | MTHFD1L | Methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1 like. (975 aa) |
| | ATP6V1A | ATPase H+ transporting V1 subunit A. (617 aa) |
| | ACSBG1 | Acyl-CoA synthetase bubblegum family member 1. (723 aa) |
| | PRKN | E3 ubiquitin-protein ligase parkin; Functions within a multiprotein E3 ubiquitin ligase complex, catalyzing the covalent attachment of ubiquitin moieties onto substrate proteins; Belongs to the RBR family. Parkin subfamily. (463 aa) |
| | CARNS1 | Carnosine synthase 1. (949 aa) |
| | PC | Pyruvate carboxylase; Catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. (1183 aa) |
| | AACS | Acetoacetyl-CoA synthetase. (674 aa) |
| | ACSL6 | Acyl-CoA synthetase long-chain family member 6 isoform a (Predicted). (723 aa) |
| | UBA7 | Ubiquitin like modifier activating enzyme 7; Belongs to the ubiquitin-activating E1 family. (1004 aa) |
| | CARS2 | Cysteinyl-tRNA synthetase 2, mitochondrial. (560 aa) |
| | FARSB | Phenylalanyl-tRNA synthetase subunit beta. (597 aa) |
| | ACSM5 | Acyl-CoA synthetase medium chain family member 5. (579 aa) |
| | LIG3 | DNA ligase. (1008 aa) |
| | TTLL1 | Tubulin tyrosine ligase like 1. (423 aa) |
| | H0X0N3_OTOGA | Uncharacterized protein. (805 aa) |
| | TTLL5 | Tubulin tyrosine ligase like 5. (1332 aa) |
| | TARS1 | Threonyl-tRNA synthetase. (723 aa) |
| | MCCC2 | Methylcrotonoyl-CoA carboxylase 2. (564 aa) |
| | TMEM179B | Transmembrane protein 179B. (219 aa) |
| | UBA2 | SUMO-activating enzyme subunit 2; The heterodimer acts as an E1 ligase for SUMO1, SUMO2, SUMO3, and probably SUMO4. It mediates ATP-dependent activation of SUMO proteins followed by formation of a thioester bond between a SUMO protein and a conserved active site cysteine residue on UBA2/SAE2. Belongs to the ubiquitin-activating E1 family. (640 aa) |
| | LARS1 | Leucyl-tRNA synthetase; Belongs to the class-I aminoacyl-tRNA synthetase family. (1143 aa) |
| | GMPS | Guanine monophosphate synthase. (595 aa) |
| | ACSS1 | Acetyl-coenzyme A synthetase. (691 aa) |
| | H0WY57_OTOGA | AA_TRNA_LIGASE_II domain-containing protein. (519 aa) |
| | AARS2 | Alanyl-tRNA synthetase 2, mitochondrial; Catalyzes the attachment of alanine to tRNA(Ala) in a two- step reaction: alanine is first activated by ATP to form Ala-AMP and then transferred to the acceptor end of tRNA(Ala). Also edits incorrectly charged tRNA(Ala) via its editing domain. Belongs to the class-II aminoacyl-tRNA synthetase family. (985 aa) |
| | UBA3 | Ubiquitin like modifier activating enzyme 3. (463 aa) |
| | VARS1 | Valyl-tRNA synthetase; Belongs to the class-I aminoacyl-tRNA synthetase family. (1264 aa) |
| | SUCLA2 | Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial; ATP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of ATP and thus represents the only step of substrate-level phosphorylation in the TCA. The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit. (463 aa) |
| | ACACB | Acetyl-CoA carboxylase beta. (2460 aa) |
| | TTL | Tubulin tyrosine ligase. (377 aa) |
| | YARS1 | Tyrosine--tRNA ligase. (528 aa) |
| | WARS2 | Tryptophanyl tRNA synthetase 2, mitochondrial; Belongs to the class-I aminoacyl-tRNA synthetase family. (331 aa) |
| | GCLC | Glutamate-cysteine ligase catalytic subunit. (637 aa) |
| | PCCB | Propionyl-CoA carboxylase subunit beta. (539 aa) |
| | SLC27A6 | Solute carrier family 27 member 6. (633 aa) |
| | RARS1 | Arginyl-tRNA synthetase; Belongs to the class-I aminoacyl-tRNA synthetase family. (660 aa) |
| | H0WTN5_OTOGA | Uncharacterized protein. (290 aa) |
| | VARS2 | Valyl-tRNA synthetase 2, mitochondrial; Belongs to the class-I aminoacyl-tRNA synthetase family. (1095 aa) |
| | CARS1 | Cysteinyl-tRNA synthetase. (830 aa) |
| | DARS1 | Aspartyl-tRNA synthetase. (501 aa) |
| | KARS1 | Lysine--tRNA ligase. (625 aa) |
| | TTLL7 | Tubulin tyrosine ligase like 7. (788 aa) |
| | CPS1 | Carbamoyl-phosphate synthase 1. (1505 aa) |
| | RTCA | RNA 3'-terminal phosphate cyclase. (365 aa) |
| | ACSF2 | Acyl-CoA synthetase family member 2. (591 aa) |
| | TARS2 | Threonyl-tRNA synthetase 2, mitochondrial. (688 aa) |
| | EPRS1 | Glutamyl-prolyl-tRNA synthetase. (1470 aa) |
| | MTHFD1 | Methylenetetrahydrofolate dehydrogenase, cyclohydrolase and formyltetrahydrofolate synthetase 1. (932 aa) |
| | MARS1 | Methionyl-tRNA synthetase; Belongs to the class-I aminoacyl-tRNA synthetase family. (905 aa) |
| | UBA5 | Ubiquitin like modifier activating enzyme 5. (404 aa) |
| | SLC27A2 | Solute carrier family 27 member 2. (619 aa) |
| | LARS2 | Leucyl-tRNA synthetase 2, mitochondrial; Belongs to the class-I aminoacyl-tRNA synthetase family. (815 aa) |
| | TTLL4 | Tubulin tyrosine ligase like 4. (1201 aa) |
| | SUCLG1 | Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial; Succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of either ATP or GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The alpha subunit of the enzyme binds the substrates coenzyme A and phosphate, while succinate binding and specificity for either ATP or GTP is provided by different beta subunits. (341 aa) |
| | QARS1 | Glutaminyl-tRNA synthetase; Belongs to the class-I aminoacyl-tRNA synthetase family. (720 aa) |
| | DALRD3 | DALR anticodon binding domain containing 3. (543 aa) |
| | LOC100950283 | AA_TRNA_LIGASE_II_ALA domain-containing protein. (412 aa) |
| | TRIM25 | Tripartite motif containing 25. (627 aa) |
| | GATC | Glutamyl-tRNA(Gln) amidotransferase subunit C, mitochondrial; Allows the formation of correctly charged Gln-tRNA(Gln) through the transamidation of misacylated Glu-tRNA(Gln) in the mitochondria. The reaction takes place in the presence of glutamine and ATP through an activated gamma-phospho-Glu-tRNA(Gln). Belongs to the GatC family. (109 aa) |
| | PAICS | AIRC domain-containing protein. (426 aa) |
| | AASDH | Aminoadipate-semialdehyde dehydrogenase. (1087 aa) |
| | ATP5F1B | ATP synthase subunit beta; Produces ATP from ADP in the presence of a proton gradient across the membrane. (529 aa) |
| | GHDC | GH3 domain containing. (530 aa) |
| | ASNS | Asparagine synthetase [glutamine-hydrolyzing]. (564 aa) |
| | HLCS | Holocarboxylase synthetase. (735 aa) |
| | HARS2 | Histidyl-tRNA synthetase 2, mitochondrial. (506 aa) |
| | HARS1 | Histidyl-tRNA synthetase. (509 aa) |
| | TTLL12 | Tubulin tyrosine ligase like 12. (645 aa) |
| | GSS | Glutathione synthetase. (474 aa) |
| | ACSS2 | Acyl-CoA synthetase short chain family member 2. (714 aa) |
| | ACSL1 | Acyl-CoA synthetase long chain family member 1. (698 aa) |
| | LRRC47 | Leucine rich repeat containing 47. (583 aa) |
| | SLC27A4 | Solute carrier family 27 member 4. (643 aa) |
| | RTCB | RNA-splicing ligase RtcB homolog; Catalytic subunit of the tRNA-splicing ligase complex that acts by directly joining spliced tRNA halves to mature-sized tRNAs by incorporating the precursor-derived splice junction phosphate into the mature tRNA as a canonical 3',5'-phosphodiester. May act as an RNA ligase with broad substrate specificity, and may function toward other RNAs. (505 aa) |
| | ACSF3 | Acyl-CoA synthetase family member 3. (576 aa) |
| | CTPS2 | CTP synthase; Catalyzes the ATP-dependent amination of UTP to CTP with either L-glutamine or ammonia as the source of nitrogen. (560 aa) |
| | NADSYN1 | Glutamine-dependent NAD(+) synthetase; In the C-terminal section; belongs to the NAD synthetase family. (734 aa) |
| | SLC27A3 | Solute carrier family 27 member 3. (670 aa) |
| | EARS2 | Glutamyl-tRNA synthetase 2, mitochondrial; Belongs to the class-I aminoacyl-tRNA synthetase family. (523 aa) |
| | FARS2 | Phenylalanyl-tRNA synthetase 2, mitochondrial. (451 aa) |
| | LIG1 | DNA ligase. (890 aa) |
| | MCCC1 | Methylcrotonoyl-CoA carboxylase 1. (654 aa) |
| | RIMKLA | Ribosomal modification protein rimK like family member A. (391 aa) |
| | SLC27A1 | Solute carrier family 27 member 1. (646 aa) |
