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| | A0A1V6RM71 | 37S ribosomal protein mrp10, mitochondrial; Involved in mitochondrial genome encoded proteins translation. (102 aa) |
| | COQ5 | 2-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrial; Methyltransferase required for the conversion of 2- polyprenyl-6-methoxy-1,4-benzoquinol (DDMQH2) to 2-polyprenyl-3-methyl- 6-methoxy-1,4-benzoquinol (DMQH2). (314 aa) |
| | A0A1V6QB01 | 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. (588 aa) |
| | A0A1V6QEF9 | Cytochrome b-c1 complex subunit 7; Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain; Belongs to the UQCRB/QCR7 family. (122 aa) |
| | BNA4 | Kynurenine 3-monooxygenase; Catalyzes the hydroxylation of L-kynurenine (L-Kyn) to form 3-hydroxy-L-kynurenine (L-3OHKyn). Required for synthesis of quinolinic acid. (511 aa) |
| | A0A1V6QH15 | Iron-sulfur cluster assembly protein; Scaffold protein for the de novo synthesis of iron-sulfur (Fe-S) clusters within mitochondria, which is required for maturation of both mitochondrial and cytoplasmic [2Fe-2S] and [4Fe-4S] proteins. (201 aa) |
| | A0A1V6QID0 | Magnesium transporter. (351 aa) |
| | PSD1 | Phosphatidylserine decarboxylase proenzyme 1, mitochondrial; Catalyzes the formation of phosphatidylethanolamine (PtdEtn) from phosphatidylserine (PtdSer). Plays a central role in phospholipid metabolism and in the interorganelle trafficking of phosphatidylserine. Belongs to the phosphatidylserine decarboxylase family. PSD-B subfamily. Eukaryotic type I sub-subfamily. (546 aa) |
| | A0A1V6QIT2 | Succinate dehydrogenase [ubiquinone] cytochrome b small subunit. (169 aa) |
| | A0A1V6QK00 | Cysteine proteinase 1, mitochondrial; Has aminopeptidase activity, shortening substrate peptides sequentially by 1 amino acid. Has bleomycin hydrolase activity, which can protect the cell from the toxic effects of bleomycin. Has homocysteine-thiolactonase activity, protecting the cell against homocysteine toxicity. (497 aa) |
| | NTH1 | Endonuclease III homolog; Bifunctional DNA N-glycosylase with associated apurinic/apyrimidinic (AP) lyase function that catalyzes the first step in base excision repair (BER), the primary repair pathway for the repair of oxidative DNA damage. The DNA N-glycosylase activity releases the damaged DNA base from DNA by cleaving the N-glycosidic bond, leaving an AP site. The AP lyase activity cleaves the phosphodiester bond 3' to the AP site by a beta-elimination. Primarily recognizes and repairs oxidative base damage of pyrimidines. (424 aa) |
| | A0A1V6QQD2 | Mitochondrial inner membrane protease ATP23; Has a dual role in the assembly of mitochondrial ATPase. Belongs to the peptidase M76 family. (239 aa) |
| | ALA1 | Alanine--tRNA ligase; 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. (910 aa) |
| | A0A1V6QRL5 | COX assembly mitochondrial protein; Required for mitochondrial cytochrome c oxidase (COX) assembly and respiration; Belongs to the CMC family. (103 aa) |
| | A0A1V6QRP6 | Letm1 RBD domain-containing protein. (381 aa) |
| | PAD1 | Flavin prenyltransferase PAD1, mitochondrial; Flavin prenyltransferase that catalyzes the synthesis of the prenylated FMN cofactor (prenyl-FMN) for the ferulic acid decarboxylase FDC1. The prenyltransferase is metal-independent and links a dimethylallyl moiety from dimethylallyl monophosphate (DMAP) to the flavin N5 and C6 atoms of FMN; Belongs to the UbiX/PAD1 family. (200 aa) |
| | A0A1V6QRT9 | CDP-diacylglycerol--glycerol-3-phosphate 3-phosphatidyltransferase; Functions in the biosynthesis of the anionic phospholipids phosphatidylglycerol and cardiolipin; Belongs to the CDP-alcohol phosphatidyltransferase class-II family. (532 aa) |
| | A0A1V6QS10 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. (135 aa) |
| | A0A1V6QSM5 | NADPH:adrenodoxin oxidoreductase, mitochondrial. (515 aa) |
| | ADK2 | GTP:AMP phosphotransferase, mitochondrial; Involved in maintaining the homeostasis of cellular nucleotides by catalyzing the interconversion of nucleoside phosphates. Has GTP:AMP phosphotransferase and ITP:AMP phosphotransferase activities. (255 aa) |
| | A0A1V6QU61 | COX assembly mitochondrial protein; Required for mitochondrial cytochrome c oxidase (COX) assembly and respiration; Belongs to the CMC family. (141 aa) |
| | MDM12 | Mitochondrial distribution and morphology protein 12; Component of the ERMES/MDM complex, which serves as a molecular tether to connect the endoplasmic reticulum (ER) and mitochondria. Components of this complex are involved in the control of mitochondrial shape and protein biogenesis, and function in nonvesicular lipid trafficking between the ER and mitochondria. MDM12 is required for the interaction of the ER-resident membrane protein MMM1 and the outer mitochondrial membrane-resident beta-barrel protein MDM10. The MDM12-MMM1 subcomplex functions in the major beta-barrel assembly pat [...] (437 aa) |
| | DRE2 | Fe-S cluster assembly protein DRE2; Component of the cytosolic iron-sulfur (Fe-S) protein assembly (CIA) machinery required for the maturation of extramitochondrial Fe-S proteins. Part of an electron transfer chain functioning in an early step of cytosolic Fe-S biogenesis, facilitating the de novo assembly of a [4Fe-4S] cluster on the scaffold complex CFD1-NBP35. Electrons are transferred to DRE2 from NADPH via the FAD- and FMN-containing protein TAH18. TAH18-DRE2 are also required for the assembly of the diferric tyrosyl radical cofactor of ribonucleotide reductase (RNR), probably by [...] (320 aa) |
| | A0A1V6QUE3 | Protein-serine/threonine kinase. (435 aa) |
| | A0A1V6QUL8 | NADH-ubiquinone oxidoreductase; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. (159 aa) |
| | TRM5 | tRNA (guanine(37)-N1)-methyltransferase; Specifically methylates the N1 position of guanosine-37 in various cytoplasmic and mitochondrial tRNAs. Methylation is not dependent on the nature of the nucleoside 5' of the target nucleoside. This is the first step in the biosynthesis of wybutosine (yW), a modified base adjacent to the anticodon of tRNAs and required for accurate decoding; Belongs to the TRM5 / TYW2 family. (480 aa) |
| | COQ4 | Ubiquinone biosynthesis protein COQ4, mitochondrial; Component of the coenzyme Q biosynthetic pathway. May play a role in organizing a multi-subunit COQ enzyme complex required for coenzyme Q biosynthesis. Required for steady-state levels of other COQ polypeptides; Belongs to the COQ4 family. (285 aa) |
| | FEN1 | Flap endonuclease 1; Structure-specific nuclease with 5'-flap endonuclease and 5'- 3' exonuclease activities involved in DNA replication and repair. During DNA replication, cleaves the 5'-overhanging flap structure that is generated by displacement synthesis when DNA polymerase encounters the 5'-end of a downstream Okazaki fragment. It enters the flap from the 5'-end and then tracks to cleave the flap base, leaving a nick for ligation. Also involved in the long patch base excision repair (LP-BER) pathway, by cleaving within the apurinic/apyrimidinic (AP) site- terminated flap. Acts as [...] (403 aa) |
| | A0A1V6QV33 | SURF1-like protein; Probably involved in the biogenesis of the COX complex. Belongs to the SURF1 family. (318 aa) |
| | A0A1V6QV84 | Cytochrome c oxidase subunit 6A, mitochondrial. (140 aa) |
| | A0A1V6QVI9 | zf-Tim10_DDP domain-containing protein; Belongs to the small Tim family. (120 aa) |
| | COQ7 | 5-demethoxyubiquinone hydroxylase, mitochondrial; Catalyzes the hydroxylation of 2-polyprenyl-3-methyl-6- methoxy-1,4-benzoquinol (DMQH2) during ubiquinone biosynthesis. Has also a structural role in the COQ enzyme complex, stabilizing other COQ polypeptides. (247 aa) |
| | A0A1V6QW15 | Letm1 RBD domain-containing protein. (541 aa) |
| | A0A1V6QW96 | Protein-serine/threonine kinase. (438 aa) |
| | PIM1 | Lon protease homolog, mitochondrial; ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix. May also have a chaperone function in the assembly of inner membrane protein complexes. Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome. Binds to mitochondrial DNA in a site-specific manner; Belongs to the peptidase S16 family. (1116 aa) |
| | A0A1V6QX51 | Sidoreflexin. (339 aa) |
| | A0A1V6QXM6 | Cytochrome b-c1 complex subunit Rieske, mitochondrial; Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis. (237 aa) |
| | UNG1 | Uracil-DNA glycosylase; Excises uracil residues from the DNA which can arise as a result of misincorporation of dUMP residues by DNA polymerase or due to deamination of cytosine. (372 aa) |
| | A0A1V6QY63 | Cytochrome c heme lyase; Links covalently the heme group to the apoprotein of cytochrome c. (329 aa) |
| | A0A1V6QYA1 | Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial; 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. (443 aa) |
| | A0A1V6QYD6 | Protein arginine methyltransferase NDUFAF7; Arginine methyltransferase involved in the assembly or stability of mitochondrial NADH:ubiquinone oxidoreductase complex (complex I). (494 aa) |
| | A0A1V6QYT8 | Mitochondrial import inner membrane translocase subunit TIM44; Essential component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner; Belongs to the Tim44 family. (507 aa) |
| | A0A1V6QZS5 | GrpE protein homolog; Essential component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner; Belongs to the GrpE family. (241 aa) |
| | A0A1V6QZV0 | Magnesium transporter. (586 aa) |
| | A0A1V6QZV2 | N-acetyltransferase domain-containing protein; In the N-terminal section; belongs to the acetylglutamate kinase family. (891 aa) |
| | A0A1V6QZY7 | Cytochrome c oxidase subunit; This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport. (92 aa) |
| | A0A1V6QZZ0 | Cytochrome c domain-containing protein; Electron carrier protein. The oxidized form of the cytochrome c heme group can accept an electron from the heme group of the cytochrome c1 subunit of cytochrome reductase. Cytochrome c then transfers this electron to the cytochrome oxidase complex, the final protein carrier in the mitochondrial electron-transport chain. (195 aa) |
| | A0A1V6R011 | Mitochondrial pyruvate carrier; Mediates the uptake of pyruvate into mitochondria. Belongs to the mitochondrial pyruvate carrier (MPC) (TC 2.A.105) family. (179 aa) |
| | ADK1 | Adenylate kinase; Catalyzes the reversible transfer of the terminal phosphate group between ATP and AMP. Plays an important role in cellular energy homeostasis and in adenine nucleotide metabolism. Adenylate kinase activity is critical for regulation of the phosphate utilization and the AMP de novo biosynthesis pathways. (257 aa) |
| | A0A1V6R0L6 | Mitochondrial glycine transporter; Mitochondrial glycine transporter that imports glycine into the mitochondrial matrix. Plays an important role in providing glycine for the first enzymatic step in heme biosynthesis, the condensation of glycine with succinyl-CoA to produce 5-aminolevulinate (ALA) in the miochondrial matrix; Belongs to the mitochondrial carrier (TC 2.A.29) family. SLC25A38 subfamily. (324 aa) |
| | A0A1V6R0Q8 | 37S ribosomal protein S25, mitochondrial. (259 aa) |
| | A0A1V6R166 | Cytochrome c heme lyase; Links covalently the heme group to the apoprotein of cytochrome c. (288 aa) |
| | A0A1V6R1E4 | Isocitrate dehydrogenase [NAD] subunit, mitochondrial. (384 aa) |
| | A0A1V6R1J0 | MICOS complex subunit MIC10; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (91 aa) |
| | A0A1V6R1Q8 | 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite; Belongs to the enoyl-CoA hydratase/isomerase family. (473 aa) |
| | A0A1V6R218 | Prohibitin. (279 aa) |
| | A0A1V6R2F0 | Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial; Iron-sulfur protein (IP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). (271 aa) |
| | A0A1V6R2Z8 | 4-hydroxybenzoate polyprenyltransferase, mitochondrial; Catalyzes the prenylation of para-hydroxybenzoate (PHB) with an all-trans polyprenyl group. Mediates the second step in the final reaction sequence of coenzyme Q (CoQ) biosynthesis, which is the condensation of the polyisoprenoid side chain with PHB, generating the first membrane-bound Q intermediate. (417 aa) |
| | AIM41 | Altered inheritance of mitochondria protein 41. (179 aa) |
| | A0A1V6R392 | zf-Tim10_DDP domain-containing protein; Belongs to the small Tim family. (91 aa) |
| | A0A1V6R3E7 | Mitochondrial Rho GTPase; Mitochondrial GTPase involved in mitochondrial trafficking. Belongs to the mitochondrial Rho GTPase family. (632 aa) |
| | A0A1V6R4A4 | MICOS complex subunit MIC12; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (125 aa) |
| | A0A1V6R545 | Cytochrome c oxidase subunit 7A; This small integral protein plays a role in holoenzyme assembly or stability. (62 aa) |
| | A0A1V6R559 | Phosphatidyl-N-methylethanolamine N-methyltransferase; Catalyzes the second two steps of the methylation pathway of phosphatidylcholine biosynthesis, the SAM-dependent methylation of phosphatidylmonomethylethanolamine (PMME) to phosphatidyldimethylethanolamine (PDME) and of PDME to phosphatidylcholine (PC). (201 aa) |
| | A0A1V6R590 | Ketol-acid reductoisomerase, mitochondrial; Belongs to the ketol-acid reductoisomerase family. (398 aa) |
| | GUF1 | Translation factor GUF1, mitochondrial; Promotes mitochondrial protein synthesis. May act as a fidelity factor of the translation reaction, by catalyzing a one-codon backward translocation of tRNAs on improperly translocated ribosomes. Binds to mitochondrial ribosomes in a GTP-dependent manner. (665 aa) |
| | A0A1V6R5L5 | Amino-acid acetyltransferase, mitochondrial; N-acetylglutamate synthase involved in arginine biosynthesis. Belongs to the acetyltransferase family. (711 aa) |
| | A0A1V6R6I2 | Lipoyl synthase, mitochondrial; Catalyzes the radical-mediated insertion of two sulfur atoms into the C-6 and C-8 positions of the octanoyl moiety bound to the lipoyl domains of lipoate-dependent enzymes, thereby converting the octanoylated domains into lipoylated derivatives. (416 aa) |
| | A0A1V6R6N2 | Altered inheritance of mitochondria protein 24, mitochondrial. (348 aa) |
| | A0A1V6R743 | Succinyl-CoA:3-ketoacid-coenzyme A transferase; Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate. (543 aa) |
| | A0A1V6R765 | 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. (500 aa) |
| | A0A1V6R784 | Isocitrate dehydrogenase [NAD] subunit, mitochondrial. (384 aa) |
| | A0A1V6R7X7 | Aconitate hydratase, mitochondrial; Belongs to the aconitase/IPM isomerase family. (798 aa) |
| | A0A1V6R819 | 5-aminolevulinate synthase; Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family. (645 aa) |
| | A0A1V6R896 | Succinate--CoA ligase [ADP-forming] subunit alpha, mitochondrial; 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 alpha subunit of the enzyme binds the substrates coenzyme A and phosphate, while succinate binding and nucleotide specificity is provided by the beta subunit. (329 aa) |
| | A0A1V6R8B7 | Glycine cleavage system P protein; The glycine cleavage system catalyzes the degradation of glycine. (1057 aa) |
| | cprA | NADPH--cytochrome P450 reductase; This enzyme is required for electron transfer from NADP to cytochrome P450 in microsomes. It can also provide electron transfer to heme oxygenase and cytochrome B5. Involved in ergosterol biosynthesis. In the C-terminal section; belongs to the flavoprotein pyridine nucleotide cytochrome reductase family. (695 aa) |
| | A0A1V6R9X8 | ATP synthase subunit d, mitochondrial; Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F(1) - containing the extramembraneous catalytic core, and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F(1) is coupled via a rotary mechanism of the cent [...] (173 aa) |
| | A0A1V6RA66 | Uncharacterized protein. (164 aa) |
| | COQ3 | Ubiquinone biosynthesis O-methyltransferase, mitochondrial; O-methyltransferase that catalyzes the 2 O-methylation steps in the ubiquinone biosynthetic pathway; Belongs to the class I-like SAM-binding methyltransferase superfamily. UbiG/COQ3 family. (359 aa) |
| | MDM34 | Mitochondrial distribution and morphology protein 34; Component of the ERMES/MDM complex, which serves as a molecular tether to connect the endoplasmic reticulum (ER) and mitochondria. Components of this complex are involved in the control of mitochondrial shape and protein biogenesis, and function in nonvesicular lipid trafficking between the ER and mitochondria. MDM34 is required for the interaction of the ER-resident membrane protein MMM1 and the outer mitochondrial membrane-resident beta-barrel protein MDM10. (559 aa) |
| | A0A1V6RBZ2 | tRNA N6-adenosine threonylcarbamoyltransferase, mitochondrial; Required for the formation of a threonylcarbamoyl group on adenosine at position 37 (t(6)A37) in mitochondrial tRNAs that read codons beginning with adenine. Probably involved in the transfer of the threonylcarbamoyl moiety of threonylcarbamoyl-AMP (TC-AMP) to the N6 group of A37. Involved in mitochondrial genome maintenance. (451 aa) |
| | A0A1V6RCW6 | NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial; Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. (495 aa) |
| | A0A1V6RDH7 | Aminomethyltransferase; The glycine cleavage system catalyzes the degradation of glycine; Belongs to the GcvT family. (483 aa) |
| | A0A1V6RDJ8 | Homoaconitase, mitochondrial; Catalyzes the reversible hydration of cis-homoaconitate to (2R,3S)-homoisocitrate, a step in the alpha-aminoadipate pathway for lysine biosynthesis. (786 aa) |
| | A0A1V6RHW9 | Mitochondrial import inner membrane translocase subunit TIM50; Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane. (512 aa) |
| | A0A1V6RHX7 | Mitochondrial GTPase 1; Mitochondrial GTPase involved in assembly of the large ribosomal subunit. Plays a role in expression of the mitochondrial translational machinery; Belongs to the TRAFAC class YlqF/YawG GTPase family. MTG1 subfamily. (308 aa) |
| | A0A1V6RIF5 | NAD(P)H-hydrate epimerase; Catalyzes the epimerization of the S- and R-forms of NAD(P)HX, a damaged form of NAD(P)H that is a result of enzymatic or heat-dependent hydration. This is a prerequisite for the S-specific NAD(P)H-hydrate dehydratase to allow the repair of both epimers of NAD(P)HX. (237 aa) |
| | PAD1-2 | Flavin prenyltransferase PAD1, mitochondrial; Flavin prenyltransferase that catalyzes the synthesis of the prenylated FMN cofactor (prenyl-FMN) for the ferulic acid decarboxylase FDC1. The prenyltransferase is metal-independent and links a dimethylallyl moiety from dimethylallyl monophosphate (DMAP) to the flavin N5 and C6 atoms of FMN; Belongs to the UbiX/PAD1 family. (203 aa) |
| | A0A1V6RJ88 | Ubiquinone biosynthesis protein; Lipid-binding protein involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration. (288 aa) |
| | A0A1V6RJE5 | Arginine biosynthesis bifunctional protein ArgJ, mitochondrial; Catalyzes two activities which are involved in the cyclic version of arginine biosynthesis: the synthesis of acetylglutamate from glutamate and acetyl-CoA, and of ornithine by transacetylation between acetylornithine and glutamate; Belongs to the ArgJ family. (460 aa) |
| | MEF2 | Ribosome-releasing factor 2, mitochondrial; Mitochondrial GTPase that mediates the disassembly of ribosomes from messenger RNA at the termination of mitochondrial protein biosynthesis. Not involved in the GTP-dependent ribosomal translocation step during translation elongation; Belongs to the TRAFAC class translation factor GTPase superfamily. Classic translation factor GTPase family. EF-G/EF-2 subfamily. (905 aa) |
| | TAH18 | NADPH-dependent diflavin oxidoreductase 1; Component of the cytosolic iron-sulfur (Fe-S) protein assembly (CIA) machinery. Required for the maturation of extramitochondrial Fe-S proteins. Part of an electron transfer chain functioning in an early step of cytosolic Fe-S biogenesis. Transfers electrons from NADPH to the Fe-S cluster of DRE2. Positively controls H(2)O(2)-induced cell death; In the C-terminal section; belongs to the flavoprotein pyridine nucleotide cytochrome reductase family. (642 aa) |
| | A0A1V6RJM2 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. (186 aa) |
| | A0A1V6RJP5 | Succinyl-CoA:3-ketoacid-coenzyme A transferase; Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate. (518 aa) |
| | A0A1V6RKF1 | Protoheme IX farnesyltransferase, mitochondrial; Converts protoheme IX and farnesyl diphosphate to heme O. Belongs to the ubiA prenyltransferase family. (534 aa) |
| | A0A1V6RKN2 | Prohibitin. (307 aa) |
| | A0A1V6RKN3 | Succinate dehydrogenase assembly factor 2, mitochondrial; Plays an essential role in the assembly of succinate dehydrogenase (SDH), an enzyme complex (also referred to as respiratory complex II) that is a component of both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain, and which couples the oxidation of succinate to fumarate with the reduction of ubiquinone (coenzyme Q) to ubiquinol. Required for flavinylation (covalent attachment of FAD) of the flavoprotein subunit of the SDH catalytic dimer. (293 aa) |
| | A0A1V6RKN7 | Altered inheritance of mitochondria protein 24, mitochondrial. (399 aa) |
| | COQ6 | Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial; FAD-dependent monooxygenase required for the C5-ring hydroxylation during ubiquinone biosynthesis. Catalyzes the hydroxylation of 3-polyprenyl-4-hydroxybenzoic acid to 3-polyprenyl- 4,5-dihydroxybenzoic acid. The electrons required for the hydroxylation reaction may be funneled indirectly from NADPH via a ferredoxin/ferredoxin reductase system to COQ6. (512 aa) |
| | A0A1V6RL69 | Aconitate hydratase, mitochondrial; Belongs to the aconitase/IPM isomerase family. (783 aa) |
| | A0A1V6RLD3 | Mitochondrial pyruvate carrier; Mediates the uptake of pyruvate into mitochondria. Belongs to the mitochondrial pyruvate carrier (MPC) (TC 2.A.105) family. (125 aa) |
| | MEF1 | Elongation factor G, mitochondrial; Mitochondrial GTPase that catalyzes the GTP-dependent ribosomal translocation step during translation elongation. During this step, the ribosome changes from the pre-translocational (PRE) to the post-translocational (POST) state as the newly formed A-site-bound peptidyl-tRNA and P-site-bound deacylated tRNA move to the P and E sites, respectively. Catalyzes the coordinated movement of the two tRNA molecules, the mRNA and conformational changes in the ribosome. (799 aa) |
| | A0A1V6RLT1 | MICOS complex subunit MIC60; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (625 aa) |
| | A0A1V6RMF8 | zf-Tim10_DDP domain-containing protein. (91 aa) |
| | PIF1 | ATP-dependent DNA helicase PIF1; DNA-dependent ATPase and 5'-3' DNA helicase required for the maintenance of both mitochondrial and nuclear genome stability. (718 aa) |
| | A0A1V6RN42 | Protein-serine/threonine kinase. (456 aa) |
| | A0A1V6RNL1 | 54S ribosomal protein L31, mitochondrial. (107 aa) |
| | A0A1V6RNR3 | Glycine cleavage system H protein; The H protein shuttles the methylamine group of glycine from the P protein to the T protein; Belongs to the GcvH family. (170 aa) |
| | A0A1V6RNR9 | NAD(P)H-hydrate epimerase; Catalyzes the epimerization of the S- and R-forms of NAD(P)HX, a damaged form of NAD(P)H that is a result of enzymatic or heat-dependent hydration. This is a prerequisite for the S-specific NAD(P)H-hydrate dehydratase to allow the repair of both epimers of NAD(P)HX. (694 aa) |
| | A0A1V6RPG4 | zf-Tim10_DDP domain-containing protein. (90 aa) |
| | MDM10 | Mitochondrial distribution and morphology protein 10; Component of the ERMES/MDM complex, which serves as a molecular tether to connect the endoplasmic reticulum and mitochondria. Components of this complex are involved in the control of mitochondrial shape and protein biogenesis and may function in phospholipid exchange. MDM10 is involved in the late assembly steps of the general translocase of the mitochondrial outer membrane (TOM complex). Functions in the TOM40-specific route of the assembly of outer membrane beta-barrel proteins, including the association of TOM40 with the recepto [...] (488 aa) |
| | A0A1V6RPM8 | Mitochondrial fission 1 protein; Has a role in mitochondrial fission. (152 aa) |
| | A0A1V6RPQ2 | Mitochondrial intermediate peptidase. (795 aa) |
| | A0A1V6RPT6 | Cytochrome c oxidase assembly protein COX20, mitochondrial; Involved in the assembly of the cytochrome c oxidase complex. Belongs to the COX20 family. (204 aa) |
| | A0A1V6RQ05 | MICOS complex subunit; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (252 aa) |
