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| | GCV2 | Glycine cleavage system P protein; The glycine cleavage system catalyzes the degradation of glycine. (999 aa) |
| | CaO19.4727 | 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. (155 aa) |
| | NTG1 | 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. (320 aa) |
| | CaO19.4468 | Succinate dehydrogenase [ubiquinone] cytochrome b small subunit. (169 aa) |
| | MRS7 | Mrs7p. (508 aa) |
| | SSC1 | Heat shock protein SSC1, mitochondrial; Required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. Constitutes the ATP-driven core of the motor and binds the precursor preprotein (By similarity). (648 aa) |
| | NFS1 | Cysteine desulfurase, mitochondrial; Catalyzes the removal of elemental sulfur from cysteine to produce alanine. It supplies the inorganic sulfur for iron-sulfur (Fe- S) clusters. Plays a role in both tRNA-processing and mitochondrial metabolism. Involved in the 2-thio-modification of both 5- carboxymethylaminomethyl-2-thiouridine in mitochondrial tRNAs and 5- methoxycarbonylmethyl-2-thiouridine (mcm5s2U) in cytoplasmic tRNAs. (488 aa) |
| | CaO19.1804 | 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 mitochondrial matrix; Belongs to the mitochondrial carrier (TC 2.A.29) family. SLC25A38 subfamily. (362 aa) |
| | AIM24 | Altered inheritance of mitochondria protein 24, mitochondrial. (484 aa) |
| | SDH7 | Succinate dehydrogenase assembly factor 3, 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. Promotes maturation of the iron-sulfur protein subunit of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants. May act together with SDHAF1. (122 aa) |
| | TIM17 | Mitochondrial import inner membrane translocase subunit TIM17; Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane. (154 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 [...] (473 aa) |
| | MCR1 | NADH-cytochrome b5 reductase 2; May mediate the reduction of outer membrane cytochrome b5. Belongs to the flavoprotein pyridine nucleotide cytochrome reductase family. (301 aa) |
| | COX2 | Cytochrome c oxidase subunit 2; Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol- cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and t [...] (262 aa) |
| | NAD6 | NADH-ubiquinone oxidoreductase chain 6; 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. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). (146 aa) |
| | NAD1 | NADH-ubiquinone oxidoreductase chain 1; 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. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). (317 aa) |
| | ATP9 | ATP synthase subunit 9, 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 centr [...] (76 aa) |
| | ATP6 | ATP synthase subunit a; 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 central stalk subuni [...] (246 aa) |
| | ATP8 | ATP synthase protein 8; 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 central stalk subuni [...] (48 aa) |
| | NAD2 | NADH-ubiquinone oxidoreductase chain 2; 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. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). (475 aa) |
| | NAD3 | NADH-ubiquinone oxidoreductase chain 3; 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. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). (129 aa) |
| | NAD4L | NADH-ubiquinone oxidoreductase chain 4L; 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. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). (84 aa) |
| | NAD5 | NADH-ubiquinone oxidoreductase chain 5; 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. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). (552 aa) |
| | NAD4 | NADH-ubiquinone oxidoreductase chain 4; 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. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). (464 aa) |
| | COX3A | Cytochrome c oxidase subunit 3; Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol- cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and t [...] (269 aa) |
| | HSP78 | Heat shock protein 78, mitochondrial; Required, in concert with mitochondrial Hsp70, for the dissociation, resolubilization and refolding of aggregates of damaged proteins in the mitochondrial matrix after heat stress. May extract proteins from aggregates by unfolding and threading them in an ATP- dependent process through the axial channel of the protein hexamer, after which they can be refolded by the Hsp70 chaperone system. Required for resumption of mitochondrial respiratory function, DNA synthesis and morphology after heat stress (By similarity). Belongs to the ClpA/ClpB family. (812 aa) |
| | URA9 | Dihydroorotate dehydrogenase (quinone), mitochondrial; In the de novo pyrimidine biosynthesis pathway, catalyzes the stereospecific oxidation of (S)-dihydroorotate to orotate with reduction of flavin and the transfer of electrons to ubiquinone, which is part of the repiratory chain. Does not use fumarate and NAD as electron acceptors. (444 aa) |
| | RSM25 | 37S ribosomal protein S25, mitochondrial; Belongs to the mitochondrion-specific ribosomal protein mS23 family. (295 aa) |
| | MSS116 | ATP-dependent RNA helicase MSS116, mitochondrial; ATP-dependent RNA helicase required for mitochondrial splicing of group I and II introns. Also required for efficient mitochondrial translation (By similarity); Belongs to the DEAD box helicase family. DDX18/HAS1 subfamily. (668 aa) |
| | CaO19.4811 | Sidoreflexin. (328 aa) |
| | CaO19.4820 | Protein arginine methyltransferase NDUFAF7; Arginine methyltransferase involved in the assembly or stability of mitochondrial NADH:ubiquinone oxidoreductase complex (complex I). (540 aa) |
| | SHY1 | SURF1-like protein; Probably involved in the biogenesis of the COX complex. Belongs to the SURF1 family. (359 aa) |
| | FMP52 | Protein FMP52, mitochondrial. (229 aa) |
| | PHB2 | Prohibitin. (303 aa) |
| | CYT2 | Cytochrome c heme lyase; Links covalently the heme group to the apoprotein of cytochrome c. (251 aa) |
| | TIM12 | Tim12p. (109 aa) |
| | COX13 | Cytochrome c oxidase subunit 6A, mitochondrial. (134 aa) |
| | CaO19.1483 | COX assembly mitochondrial protein; Required for mitochondrial cytochrome c oxidase (COX) assembly and respiration; Belongs to the CMC family. (131 aa) |
| | CaO19.1485 | 54S ribosomal protein L31, mitochondrial. (113 aa) |
| | AIM36 | Altered inheritance of mitochondria protein 36, mitochondrial. (292 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. (761 aa) |
| | COX19 | Cytochrome c oxidase assembly protein COX19; Required for the assembly of mitochondrial cytochrome c oxidase; Belongs to the COX19 family. (132 aa) |
| | GCV3 | 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. (177 aa) |
| | LCL3 | Probable endonuclease LCL3. (235 aa) |
| | HER2 | 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. (450 aa) |
| | SDH4 | Succinate dehydrogenase [ubiquinone] cytochrome b small subunit. (164 aa) |
| | CaO19.1625 | 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. (140 aa) |
| | NCE103 | Carbonic anhydrase; Catalyzes the reversible hydration of CO(2) to H(2)CO(3). The main role may be to provide inorganic carbon for the bicarbonate- dependent carboxylation reactions catalyzed by pyruvate carboxylase, acetyl-CoA carboxylase and carbamoyl-phosphate synthetase. Involved in protection against oxidative damage. Acts as a CO(2) chemosensor and induces CO(2)-mediated filamentation. Essential for pathological growth in niches where sufficient CO(2) is not supplied by the host. Necessary for white-to-opaque switching at low CO(2) concentrations. (281 aa) |
| | TOM20 | Tom20p. (171 aa) |
| | MGM101 | Mitochondrial genome maintenance protein MGM101; Performs an essential function in the repair of oxidatively damaged mtDNA that is required for the maintenance of the mitochondrial genome. Binds to DNA (By similarity); Belongs to the MGM101 family. (275 aa) |
| | CaO19.2963 | NAD-dependent protein deacylase; NAD-dependent lysine demalonylase, desuccinylase and deglutarylase that specifically removes malonyl, succinyl and glutaryl groups on target proteins. Has weak NAD-dependent protein deacetylase activity; however this activity may not be physiologically relevant in vivo; Belongs to the sirtuin family. Class III subfamily. (306 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. (323 aa) |
| | CaO19.3022 | 37S ribosomal protein S24, mitochondrial; Involved in mitochondrial genome encoded proteins translation; Belongs to the mitochondrion-specific ribosomal protein mS35 family. (310 aa) |
| | EHD3 | 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite; Belongs to the enoyl-CoA hydratase/isomerase family. (502 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. (480 aa) |
| | IMP1 | Mitochondrial inner membrane protease subunit. (183 aa) |
| | MGR1 | Mitochondrial inner membrane i-AAA protease complex subunit MGR1; Component of the mitochondrial inner membrane i-AAA protease complex required for mitochondrial inner membrane protein turnover. (368 aa) |
| | CaO19.6461 | Cytochrome c oxidase assembly protein COX20, mitochondrial; Involved in the assembly of the cytochrome c oxidase complex. Belongs to the COX20 family. (171 aa) |
| | ECM42 | 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. (439 aa) |
| | ISU1 | 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. (187 aa) |
| | FIS1 | Mitochondrial fission 1 protein; Has a role in mitochondrial fission. Has a role in outer membrane fission but not matrix separation (By similarity). (154 aa) |
| | TIM13 | Mitochondrial import inner membrane translocase subunit TIM13; Mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane. Also required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space. The TIM8- TIM13 complex is non essential and only mediates [...] (108 aa) |
| | CCP1 | Cytochrome c peroxidase, mitochondrial; Destroys radicals which are normally produced within the cells and which are toxic to biological systems. (366 aa) |
| | PAM17 | Presequence translocated-associated motor subunit PAM17, mitochondrial; 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. (185 aa) |
| | OXR1 | Oxidation resistance protein 1; May be involved in protection from oxidative damage. Belongs to the OXR1 family. (345 aa) |
| | CAF17 | Putative transferase CAF17, mitochondrial. (469 aa) |
| | GEP3 | Genetic interactor of prohibitins 3, mitochondrial; May be involved in the mitochondrial lipid metabolism. Belongs to the TRAFAC class YlqF/YawG GTPase family. GEP3 subfamily. (629 aa) |
| | RSM10 | 37S ribosomal protein S10, mitochondrial; Involved in mitochondrial genome encoded proteins translation. Involved in the binding of tRNA to the ribosomes (By similarity). (234 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 N-terminal section; belongs to the flavodoxin family. (589 aa) |
| | COX16 | Cytochrome c oxidase assembly protein COX16, mitochondrial; Required for the assembly of the mitochondrial respiratory chain complex IV (CIV), also known as cytochrome c oxidase. May participate in merging the COX1 and COX2 assembly lines. Belongs to the COX16 family. (128 aa) |
| | QCR7 | 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. (127 aa) |
| | GEM1 | Mitochondrial Rho GTPase 1; Mitochondrial GTPase involved in mitochondrial trafficking. Probably involved in control of anterograde transport of mitochondria and their subcellular distribution. (716 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 (By similarity). Important for virulence. (590 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. (807 aa) |
| | PET112 | 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). (488 aa) |
| | MGE1 | 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. (242 aa) |
| | MRS2 | Mitochondrial inner membrane magnesium transporter MRS2; High-conductance magnesium-selective channel that mediates the influx of magnesium into the mitochondrial matrix. Essential for the splicing of mRNA group II introns in mitochondria by affecting mitochondrial magnesium concentrations, which are critical for group II intron splicing. It also suppresses a variety of mitochondrial intron mutations and its absence may disturb the assembly of mitochondrial membrane complexes. (468 aa) |
| | AIM9 | Altered inheritance of mitochondria protein 9, mitochondrial. (624 aa) |
| | FYV4 | Protein FYV4, mitochondrial; Involved in telomere length regulation; Belongs to the mitochondrion-specific ribosomal protein mS41 family. (170 aa) |
| | LSC1 | 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. (323 aa) |
| | CCM1 | Mitochondrial group I intron splicing factor CCM1; RNA-binding protein involved in the specific removal of group I introns in mitochondrial encoded transcripts. Maintains the stability of the small subunit mitochondrial 15S rRNA and thus the expression of the mitochondrial genome (By similarity); Belongs to the CCM1 family. (768 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. (969 aa) |
| | MRPL4 | 54S ribosomal protein L4, mitochondrial; Belongs to the universal ribosomal protein uL29 family. (309 aa) |
| | COX23 | Cytochrome c oxidase-assembly factor COX23, mitochondrial; Required for the assembly of cytochrome c oxidase. Belongs to the COX23 family. (139 aa) |
| | MZM1 | Mitochondrial zinc maintenance protein 1, mitochondrial; Assembly factor required for Rieske Fe-S protein RIP1 incorporation into the cytochrome b-c1 (CIII) complex. Functions as a chaperone, binding to this subunit within the mitochondrial matrix and stabilizing it prior to its translocation and insertion into the late CIII dimeric intermediate within the mitochondrial inner membrane. Modulates the mitochondrial matrix zinc pool (By similarity). (117 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. (456 aa) |
| | TIM44 | 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. (424 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. (1078 aa) |
| | LAP3 | 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. (509 aa) |
| | RAD27 | 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 [...] (372 aa) |
| | LYS4 | Homoaconitase, mitochondrial; Catalyzes the reversible hydration of cis-homoaconitate to (2R,3S)-homoisocitrate, a step in the alpha-aminoadipate pathway for lysine biosynthesis. (684 aa) |
| | ATG27 | Autophagy-related protein 27; Effector of VPS34 phosphatidylinositol 3-phosphate kinase signaling. Regulates the cytoplasm to vacuole transport (Cvt) vesicle formation. Plays a role in ATG protein retrieval from the pre- autophagosomal structure (PAS) and is especially required for autophagy-dependent cycling of ATG9 (By similarity); Belongs to the ATG27 family. (252 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. (249 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. (327 aa) |
| | MRPL51 | 54S ribosomal protein L51, mitochondrial; Belongs to the mitochondrion-specific ribosomal protein mL43 family. (140 aa) |
| | CaO19.6435 | Mitochondrial pyruvate carrier; Mediates the uptake of pyruvate into mitochondria. Belongs to the mitochondrial pyruvate carrier (MPC) (TC 2.A.105) family. (132 aa) |
| | CYM1 | Mitochondrial presequence protease; ATP-independent protease that degrades mitochondrial transit peptides after their cleavage. Also degrades other unstructured peptides (By similarity); Belongs to the peptidase M16 family. PreP subfamily. (1034 aa) |
| | DML1 | Protein DML1; Involved in the partitioning of the mitochondrial organelle and mitochondrial DNA (mtDNA) inheritance; Belongs to the misato family. (573 aa) |
| | RMD9 | Protein RMD9, mitochondrial; Involved in the processing or stability of mitochondrial mRNAs. Required for meiotic nuclear division (By similarity). Belongs to the RMD9 family. (613 aa) |
| | CaO19.415 | 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. (248 aa) |
| | MHR1 | Mitochondrial homologous recombination protein 1; Transcription factor involved in regulation of RNA polymerase II-dependent transcription. Also involved in regulation of mitochondrial DNA recombination, maintenance and repair, and generation of homoplasmic cells (By similarity); Belongs to the mitochondrion-specific ribosomal protein mL67 family. (239 aa) |
| | CaO19.2821 | 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. (175 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 [...] (409 aa) |
| | PUS5 | 21S rRNA pseudouridine(2819) synthase; Pseudouridylate synthase responsible for the pseudouridine- 2819 formation in mitochondrial 21S rRNA. May modulate the efficiency or the fidelity of the mitochondrial translation machinery. (248 aa) |
| | SHE9 | Sensitive to high expression protein 9 homolog, mitochondrial; Required for the maintenance of the structure of the mitochondrial inner membrane. Involved in mitochondrial morphology. Causes growth arrest when highly overexpressed (By similarity). (524 aa) |
| | PRP13 | Mitochondrial escape protein 2; Plays a role in maintaining the mitochondrial genome and in controlling the mtDNA escape. Involved in the regulation of mtDNA nucleotide structure and number. May have a dispensable role in early maturation of pre-rRNA (By similarity); Belongs to the YME2 family. (867 aa) |
| | MIC60 | 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. Plays a role in keeping cristae membranes connected to the inner boundary membrane. Also promotes protein import via the mitochondrial intermembrane space assembly (MIA) pathway (By similarity). (567 aa) |
| | DEM1 | Exonuclease V, mitochondrial; Single strand DNA specific 5' exonuclease involved in mitochondrial DNA replication and recombination. Releases dinucleotides as main products of catalysis. Has the capacity to slide across 5'double-stranded DNA or 5'RNA sequences and resumes cutting two nucleotides downstream of the double-stranded-to-single-stranded junction or RNA-to-DNA junction, respectively (By similarity). (628 aa) |
| | PAM16 | Mitochondrial import inner membrane translocase subunit TIM16; 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. In the complex, it is required to regulate activity of mtHSP70 (SSC1) via its interaction with PAM18/TIM14. May act by positioning PAM18/TIM14 in juxtaposition to mtHSP70 at the translocon to maximize ATPase stimulation (By similarity); Belongs to the TIM16/PAM16 family. (121 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). (306 aa) |
| | ATP23 | Mitochondrial inner membrane protease ATP23; Has a dual role in the assembly of mitochondrial ATPase. Acts as a protease that removes N-terminal residues of mitochondrial ATPase CF(0) subunit 6 at the intermembrane space side. Also involved in the correct assembly of the membrane-embedded ATPase CF(0) particle, probably mediating association of subunit 6 with the subunit 9 ring (By similarity); Belongs to the peptidase M76 family. (238 aa) |
| | CaO19.2281 | 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. (500 aa) |
| | AIM23 | Altered inheritance of mitochondria protein 23, mitochondrial. (429 aa) |
| | TIM23 | Mitochondrial import inner membrane translocase subunit TIM23; Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane. (218 aa) |
| | GTF1 | Glutamyl-tRNA(Gln) amidotransferase subunit F, 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). Required for proper protein synthesis within the mitochondrion; Belongs to the GatF family. (165 aa) |
| | TIM50 | 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. Required to direct preproteins in transit and direct them to the channel protein TIM23, and possibly facilitates transfer of the translocating proteins from the TOM complex to the TIM23 complex (By similarity). (469 aa) |
| | CaO19.3679 | 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. (258 aa) |
| | TIM21 | Mitochondrial import inner membrane translocase subunit TIM21; Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane. Required to keep the TOM and the TIM23 complexes in close contact. At some point, it is released from the TOM23 complex to allow protein translocation into the mitochondrial matrix (By similarity). (268 aa) |
| | ATP25 | ATPase synthesis protein 25, mitochondrial; Probable mitochondrial mRNA stabilization factor. Belongs to the ATP25 family. (580 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 class I-like SAM-binding methyltransferase superfamily. TRM5/TYW2 family. (449 aa) |
| | NDH51 | 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. (490 aa) |
| | SUV3 | ATP-dependent RNA helicase SUV3, mitochondrial; Required for intron-independent turnover and processing of mitochondrial RNA. It is a key control element in nuclear-mitochondrial interactions (By similarity). Required for embedded hyphal growth, for wild-type respiratory growth, and biofilm development. Required for chlamydospore formation, distinctive morphological feature of the fungal pathogen C.albicans that can be induced to form in oxygen- limited environments and has been reported in clinical specimens. Plays am important role in virulence. (720 aa) |
| | PHB1 | Prohibitin. (283 aa) |
| | PAM18 | Mitochondrial import inner membrane translocase subunit TIM14; 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. In the complex, it is required to stimulate activity of mtHSP70 (SSC1) (By similarity); Belongs to the TIM14 family. (157 aa) |
| | LPE10 | Mitochondrial inner membrane magnesium transporter LPE10; Mitochondrial inner membrane magnesium transporter required for mitochondrial magnesium homeostasis. Modulates the conductance of the MRS2 channel. Involved in the splicing of mRNA group II introns in mitochondria by affecting mitochondrial magnesium concentrations, which are critical for group II intron splicing. (453 aa) |
| | MRH4 | ATP-dependent RNA helicase MRH4, mitochondrial; ATP-binding RNA helicase involved in mitochondrial RNA metabolism. Required for maintenance of mitochondrial DNA (By similarity); Belongs to the DEAD box helicase family. MRH4 subfamily. (555 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 [...] (428 aa) |
| | HXK1 | N-acetylglucosamine kinase 1; Component of the N-acetylglucosamine catabolic cascade that phosphorylates N-acetylglucosamine (GlcNAc), and allows the unique ability to utilise GlcNAc as carbon source. Converts GlcNAc to GlcNAc- 6-P. Also able to phosphorylate glucose, glucosamine (GlcN), and mannose. Galactose, fructose, N-acetylmannosamine (ManNAc), mannosamine (ManN), galactosamine (GalN), and N-acetylgalactosamine (GalNAc) are not phosphorylated by HXK1. GlcNAc metabolism is closely associated with virulence and morphogenesis, and is involved in the cell wall synthesis. Acts both as [...] (493 aa) |
| | CaO19.6132 | 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. (127 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. Efficiently unwinds G-quadruplex (G4) DNA structures and forked RNA-DNA hybrids. Resolves G4 structures, preventing replication pausing and double-strand breaks (DSBs) at G4 motifs. Involved in the maintenance of telomeric DNA. Inhibits telomere elongation, de novo telomere formation and telomere addition to DSBs via catalytic inhibition of telomerase. Reduces the processivity of telomerase by displacing active telomerase from DNA [...] (906 aa) |
| | ARG2 | Amino-acid acetyltransferase, mitochondrial; N-acetylglutamate synthase involved in arginine biosynthesis. (580 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. (623 aa) |
| | TIM8 | Mitochondrial import inner membrane translocase subunit TIM8; Mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane. Also required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space. The TIM8- TIM13 complex is non essential and only mediates [...] (88 aa) |
| | RCF1 | Respiratory supercomplex factor 1, mitochondrial; Cytochrome c oxidase subunit which plays a role in assembly of respiratory supercomplexes. (155 aa) |
| | CBR1 | NADH-cytochrome b5 reductase 1; Electron donor reductase for cytochrome b5. The cytochrome b5/NADH cytochrome b5 reductase electron transfer system supports the catalytic activity of several sterol biosynthetic enzymes (By similarity). (294 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. Belongs to the TRAFAC class translation factor GTPase superfamily. Classic translation factor GTPase family. LepA subfamily. (654 aa) |
| | ATP7 | 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 [...] (174 aa) |
| | TPC1 | Mitochondrial thiamine pyrophosphate carrier 1; Mitochondrial transporter that mediates uptake of thiamine pyrophosphate (ThPP) into mitochondria; Belongs to the mitochondrial carrier (TC 2.A.29) family. (301 aa) |
| | SYM1 | Protein SYM1; May be involved in cellular response to stress. Required to maintain mitochondrial DNA (mtDNA) integrity and stability (By similarity); Belongs to the peroxisomal membrane protein PXMP2/4 family. (195 aa) |
| | CBP4 | Assembly factor CBP4; Essential for the assembly of ubiquinol-cytochrome c reductase. It has a direct effect on the correct occurrence of the Rieske protein, core 4, core 5 and apocytochrome b (By similarity). Belongs to the CBP4 family. (144 aa) |
| | SDH2 | 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). (263 aa) |
| | ATM1 | Iron-sulfur clusters transporter ATM1, mitochondrial; Performs an essential function in the generation of cytoplasmic iron-sulfur proteins by mediating the ATP-dependent export of Fe/S cluster precursors synthesized by NFS1 and other mitochondrial proteins. Hydrolyzes ATP. Binds glutathione and may function by transporting a glutathione-conjugated iron-sulfur compound (By similarity). (750 aa) |
| | TIM9 | Mitochondrial import inner membrane translocase subunit TIM9; Mitochondrial intermembrane chaperone that participates in the import and insertion of multi-pass transmembrane proteins into the mitochondrial inner membrane. Also required for the transfer of beta- barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space (By similarity); Belongs to the small Tim family. (87 aa) |
| | OCT1 | Mitochondrial intermediate peptidase; Cleaves proteins, imported into the mitochondrion, to their mature size. While most mitochondrial precursor proteins are processed to the mature form in one step by mitochondrial processing peptidase (MPP), the sequential cleavage by MIP of an octapeptide after initial processing by MPP is a required step for a subgroup of nuclear-encoded precursor proteins destined for the matrix or the inner membrane (By similarity). (783 aa) |
| | QCR2 | Cytochrome b-c1 complex subunit 2, mitochondrial; Component of the ubiquinol-cytochrome c oxidoreductase, a multisubunit transmembrane complex that is part of the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradie [...] (374 aa) |
| | POR1 | Mitochondrial outer membrane protein porin; Forms a channel through the cell membrane that allows diffusion of small hydrophilic molecules. The channel adopts an open conformation at low or zero membrane potential and a closed conformation at potentials above 30-40 mV. The open state has a weak anion selectivity whereas the closed state is cation-selective (By similarity); Belongs to the eukaryotic mitochondrial porin family. (282 aa) |
| | ACO1 | Aconitate hydratase, mitochondrial; Catalyzes the isomerization of citrate to isocitrate via cis- aconitate, a step in the citric acid cycle; Belongs to the aconitase/IPM isomerase family. (777 aa) |
| | CYC1 | Cytochrome c; 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. (110 aa) |
| | TIM54 | Mitochondrial import inner membrane translocase subunit TIM54; Essential component of the TIM22 complex, a complex that mediates the import and insertion of multi-pass transmembrane proteins into the mitochondrial inner membrane. The TIM22 complex forms a twin- pore translocase that uses the membrane potential as external driving force (By similarity). (400 aa) |
| | LAB5 | 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. (386 aa) |
| | COB | Cytochrome b; Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex) that is part of the mitochondrial respiratory chain. The b-c1 complex mediates electron transfer from ubiquinol to cytochrome c. Contributes to the generation of a proton gradient across the mitochondrial membrane that is then used for ATP synthesis. (387 aa) |
| | COX1 | Cytochrome c oxidase subunit 1; Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol- cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and t [...] (531 aa) |
| | MRPS9 | 37S ribosomal protein S9, mitochondrial. (336 aa) |
| | HEM1 | 5-aminolevulinate synthase, mitochondrial; Catalyzes the synthesis of 5-aminolevulinate (ALA) from succinyl-CoA and glycine, the first and rate-limiting step in heme biosynthesis; Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family. (564 aa) |
| | MIA40 | Mitochondrial intermembrane space import and assembly protein 40; Required for the import and folding of small cysteine- containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Forms a redox cycle with ERV1 that involves a disulfide relay system. Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria. The oxidized form of MIA40 forms a transient intermolecular disulfide bridge with the reduced precursor protein, resulting in oxidation of the precursor protein that now contains an intramolecular disulfide bond [...] (252 aa) |
| | ALO1 | D-arabinono-1,4-lactone oxidase; Belongs to the oxygen-dependent FAD-linked oxidoreductase family. (557 aa) |
| | HSP60 | Heat shock protein 60, mitochondrial; May participate in assembly and/or disassembly of proteins imported into the mitochondrion. HSP60 are ATPases and have affinity for unfolded proteins (By similarity); Belongs to the chaperonin (HSP60) family. (566 aa) |
| | COQ2 | 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. (348 aa) |
| | CaO19.6602 | 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. (289 aa) |
| | PDK2 | Protein-serine/threonine kinase. (511 aa) |
| | CaO19.6152 | Protein arginine methyltransferase NDUFAF7; Arginine methyltransferase involved in the assembly or stability of mitochondrial NADH:ubiquinone oxidoreductase complex (complex I). (527 aa) |
| | LSC2 | 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. (415 aa) |
| | ACO2 | Aconitate hydratase, mitochondrial; Belongs to the aconitase/IPM isomerase family. (791 aa) |
| | A0A1D8PRB8 | COX assembly mitochondrial protein; Required for mitochondrial cytochrome c oxidase (COX) assembly and respiration; Belongs to the CMC family. (99 aa) |
| | PEL1 | 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. (501 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. (222 aa) |
| | A0A1D8PQD5 | 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. (83 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. (229 aa) |
| | GCV1 | Aminomethyltransferase; The glycine cleavage system catalyzes the degradation of glycine; Belongs to the GcvT family. (394 aa) |
| | ILV5 | Ketol-acid reductoisomerase, mitochondrial; Belongs to the ketol-acid reductoisomerase family. (400 aa) |
| | A0A1D8PNP0 | 37S ribosomal protein mrp10, mitochondrial; Involved in mitochondrial genome encoded proteins translation. (95 aa) |
| | CaO19.3167 | Protoheme IX farnesyltransferase, mitochondrial; Converts protoheme IX and farnesyl diphosphate to heme O. Belongs to the ubiA prenyltransferase family. (461 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. (246 aa) |
| | CYC3 | Cytochrome c heme lyase; Links covalently the heme group to the apoprotein of cytochrome c. (264 aa) |
| | IMP2 | Mitochondrial inner membrane protease subunit. (162 aa) |
| | CaO19.5694 | Genetic interactor of prohibitin 5, mitochondrial; Essential for respiratory growth and required for maintenance of mtDNA. Required for cell survival in the absence of prohibitins. Belongs to the GEP5 family. (232 aa) |
| | A0A1D8PM81 | 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. (87 aa) |
| | CaO19.1440 | Altered inheritance of mitochondria protein 24, mitochondrial. (371 aa) |
| | NCP1 | 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. (680 aa) |
| | TIM10 | Protein transporter. (91 aa) |
| | QRI7 | 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. (450 aa) |
| | OPI3 | 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). (225 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. (355 aa) |
| | RIP1 | 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. (213 aa) |
| | CaO19.357 | Prohibitin. (283 aa) |
| | CaO19.252 | Mitochondrial pyruvate carrier; Mediates the uptake of pyruvate into mitochondria. Belongs to the mitochondrial pyruvate carrier (MPC) (TC 2.A.105) family. (117 aa) |
| | TIM22 | Mitochondrial import inner membrane translocase subunit TIM22; Essential core component of the TIM22 complex, a complex that mediates the import and insertion of multi-pass transmembrane proteins into the mitochondrial inner membrane. In the TIM22 complex, it constitutes the voltage-activated and signal-gated channel. Forms a twin-pore translocase that uses the membrane potential as external driving force in 2 voltage-dependent steps (By similarity). (184 aa) |
| | COX9 | Cytochrome c oxidase subunit 7A; This small integral protein plays a role in holoenzyme assembly or stability. (61 aa) |
| | IDH2 | Isocitrate dehydrogenase [NAD] subunit, mitochondrial. (369 aa) |
| | CaO19.1140 | 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. (208 aa) |
| | IDH1 | Isocitrate dehydrogenase [NAD] subunit, mitochondrial. (364 aa) |
| | ARG5,6 | Bifunctional acetylglutamate kinase/N-acetyl-gamma-glutamyl-phosphate reductase; In the N-terminal section; belongs to the acetylglutamate kinase family. (857 aa) |
