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| | 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. (270 aa) |
| | pet117 | Protein pet117, mitochondrial; Involved in the assembly of cytochrome c oxidase. Belongs to the PET117 family. (88 aa) |
| | sdh8 | Succinate dehydrogenase assembly factor 4, 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. Binds to the flavoprotein subunit sdh1 in its FAD-bound form, blocking the generation of excess reactive oxigen species (ROS) and facilitating its assembly with the iron [...] (100 aa) |
| | mrp49 | Probable 54S ribosomal protein MRP49, mitochondrial; Involved in mitochondrial translation. (135 aa) |
| | fmc1 | ATP synthase assembly factor fmc1, mitochondrial; Needed for the assembly of the mitochondrial F1-F0 complex at high temperature; Belongs to the FMC1 family. (104 aa) |
| | new15 | Putative mitochondrial ribosomal protein new15, mitochondrial. (86 aa) |
| | kgd4 | 37S ribosomal protein YMR-31, mitochondrial. (89 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). (100 aa) |
| | cox20 | Cytochrome c oxidase assembly protein cox20, mitochondrial; Involved in the assembly of the cytochrome oxidase complex. Required for the maturation and subsequent assembly of the mitochondrially encoded cox2, the precursor of subunit 2 of cytochrome oxidase (By similarity); Belongs to the COX20 family. (120 aa) |
| | tam9 | 54S ribosomal protein L36, mitochondrial. (86 aa) |
| | dld1 | Dihydrolipoyl dehydrogenase, mitochondrial; Lipoamide dehydrogenase is a component of the alpha-ketoacid dehydrogenase complexes (By similarity). Malfunction of this protein blocks the progression of cell cycle from G1 to S phase; Belongs to the class-I pyridine nucleotide-disulfide oxidoreductase family. (511 aa) |
| | mrps28 | Probable 37S ribosomal protein S28, mitochondrial; Essential component of the small mitochondrial ribosomal subunit; Belongs to the universal ribosomal protein uS15 family. (288 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. (535 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). (452 aa) |
| | ips1 | Protein MTO1 homolog, mitochondrial; Involved in the 5-carboxymethylaminomethyl modification (mnm(5)s(2)U34) of the wobble uridine base in mitochondrial tRNAs. Belongs to the MnmG family. (666 aa) |
| | idh1 | Isocitrate dehydrogenase [NAD] subunit 1, mitochondrial; Performs an essential role in the oxidative function of the citric acid cycle. Also binds RNA; specifically to the 5'-untranslated leaders of mitochondrial mRNAs. (356 aa) |
| | mct1 | Malonyl CoA-acyl carrier protein transacylase, mitochondrial; Involved in biosynthesis of fatty acids in mitochondria. Belongs to the FabD family. (318 aa) |
| | mpd1 | Thioredoxin domain-containing protein C13F5.05, mitochondrial. (363 aa) |
| | rsm7 | 37S ribosomal protein S7, mitochondrial. (259 aa) |
| | SPAC16E8.17c | 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. (331 aa) |
| | SPAC17A2.11 | Uncharacterized protein C17A2.11, mitochondrial. (217 aa) |
| | coq9 | Ubiquinone biosynthesis protein coq9, mitochondrial; Lipid-binding protein involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration. (250 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. (652 aa) |
| | mrps18 | Putative 37S ribosomal protein S18, mitochondrial; Belongs to the universal ribosomal protein uS11 family. (223 aa) |
| | fmp40 | Protein adenylyltransferase SelO, mitochondrial; Catalyzes the transfer of adenosine 5'-monophosphate (AMP) to Tyr residues of target mitochondrial proteins (AMPylation). Involved in redox homeostasis by regulating the cellular response to oxidative stress. Regulates protein S-glutathionylation levels possibly by AMPylation of deglutathionylation enzymes such as glutaredoxins. (568 aa) |
| | mdm28 | LETM1 domain-containing protein mdm28, mitochondrial; Involved in mitochondrial potassium homeostasis through the mitochondrial K(+)/H(+) exchange regulation. (485 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. (789 aa) |
| | SPAC24C9.09 | Probable threonine--tRNA ligase, mitochondrial; Belongs to the class-II aminoacyl-tRNA synthetase family. (474 aa) |
| | mrp4 | 37S ribosomal protein mrp4, mitochondrial; Belongs to the universal ribosomal protein uS2 family. (263 aa) |
| | rpo41 | DNA-directed RNA polymerase, mitochondrial; DNA-dependent RNA polymerase catalyzes the transcription of DNA into RNA using the four ribonucleoside triphosphates as substrates. Combines in the mitochondrion with mitochondrial transcription factor mtf1 as a holoenzyme to recognize and initiate transcription at the core mitochondrial promoters. (1154 aa) |
| | msm1 | Probable methionine--tRNA ligase, mitochondrial; Belongs to the class-I aminoacyl-tRNA synthetase family. (539 aa) |
| | mrps9 | 37S ribosomal protein S9, mitochondrial. (271 aa) |
| | SPAC2C4.09 | Protein fmp32, mitochondrial. (216 aa) |
| | cox19 | Cytochrome c oxidase assembly protein cox19, mitochondrial; Required for the assembly of mitochondrial cytochrome c oxidase; Belongs to the COX19 family. (112 aa) |
| | rim1 | Single-stranded DNA-binding protein rim1, mitochondrial; This protein binds preferentially and cooperatively to ss- DNA. Involved in mitochondrial DNA replication (By similarity). (150 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. (558 aa) |
| | gcv1 | Probable aminomethyltransferase, mitochondrial; The glycine cleavage system catalyzes the degradation of glycine; Belongs to the GcvT family. (387 aa) |
| | nde1 | Probable NADH-ubiquinone oxidoreductase C3A11.07, mitochondrial; Catalyzes the oxidation of NADH. (551 aa) |
| | vrs2 | Valine--tRNA ligase, mitochondrial; Belongs to the class-I aminoacyl-tRNA synthetase family. (950 aa) |
| | mrpl40 | 54S ribosomal protein L40, mitochondrial; Involved in mitochondrial genome encoded proteins translation. (279 aa) |
| | mrpl28 | 54S ribosomal protein L28, mitochondrial; Involved in mitochondrial genome encoded proteins translation. (118 aa) |
| | mrps12 | Probable 37S ribosomal protein S12, mitochondrial. (162 aa) |
| | rrg9 | Required for respiratory growth protein 9, mitochondrial; Required for respiratory activity and maintenance and expression of the mitochondrial genome; Belongs to the RRG9 family. (117 aa) |
| | cld1 | Probable cardiolipin-specific deacylase, mitochondrial; Mitochondrial cardiolipin-specific phospholipase which deacylates de novo synthesized cardiolipin. Part of the remodeling process of cardiolipin, which involves deacylation-reacylation of premature cardiolipin (By similarity). (428 aa) |
| | idp1 | Probable isocitrate dehydrogenase [NADP], mitochondrial. (439 aa) |
| | ppr4 | Pentatricopeptide repeat-containing protein 4, mitochondrial; Mitochondrial RNA-binding protein that specifically activates the translation of the cox1 mRNA. (931 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. Also has an indirect role in vacuolar function where it is required for fluid-phase endocytosis. (693 aa) |
| | oxa101 | Mitochondrial inner membrane protein oxa1-1; Required for the insertion of integral membrane proteins into the mitochondrial inner membrane. Essential for the activity and assembly of cytochrome c oxidase. Not essential for viability, while oxa102 is essential. When both genes are deleted the cell is non- viable, suggesting that oxa101 act as a back-up for oxa102. Belongs to the OXA1/ALB3/YidC family. (374 aa) |
| | mug100 | Meiotically up-regulated gene 100 protein, mitochondrial; Has a role in meiosis. (313 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). Together with psd2 and psd3, responsible for the majority of phosphatidylethanolamine synthesis. Belongs to the phosphatidylserine decarboxylase family. PSD-B subfamily. Eukaryotic type I sub-subfamily. (437 aa) |
| | mrpl7 | Probable 54S ribosomal protein L7, mitochondrial. (287 aa) |
| | mrpl35 | 54S ribosomal protein L35, mitochondrial; Belongs to the phosphatidylethanolamine-binding protein family. Mitochondrion-specific ribosomal protein mL38 subfamily. (308 aa) |
| | rsm27 | 37S ribosomal protein S27, mitochondrial; Involved in mitochondrial genome encoded proteins translation. (93 aa) |
| | eca39 | Branched-chain-amino-acid aminotransferase, mitochondrial; Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine; Belongs to the class-IV pyridoxal-phosphate-dependent aminotransferase family. (427 aa) |
| | gut2 | Glycerol-3-phosphate dehydrogenase, mitochondrial; Belongs to the FAD-dependent glycerol-3-phosphate dehydrogenase family. (649 aa) |
| | mrp51 | 37S ribosomal protein mrp51, mitochondrial. (303 aa) |
| | msw1 | Tryptophan--tRNA ligase, mitochondrial. (361 aa) |
| | ppr7 | Pentatricopeptide repeat-containing protein 7, mitochondrial; Mitochondrial RNA-binding protein required for the stability of the atp6 mRNA. (658 aa) |
| | ppr3 | Pentatricopeptide repeat-containing protein 3, mitochondrial; 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. (687 aa) |
| | mmf1 | Protein mmf1, mitochondrial; Plays a role in the maintenance of mitochondrial DNA. Belongs to the RutC family. (162 aa) |
| | ade9 | C-1-tetrahydrofolate synthase, mitochondrial; In the C-terminal section; belongs to the formate-- tetrahydrofolate ligase family. (972 aa) |
| | hrs1 | Histidine--tRNA ligase, mitochondrial; Catalyzes the aminoacylation of histidyl-tRNA in both the cytoplasm and the mitochondrion. (563 aa) |
| | mrpl3 | 54S ribosomal protein L3, mitochondrial; Belongs to the ribonuclease III family. Mitochondrion- specific ribosomal protein mL44 subfamily. (326 aa) |
| | mge1 | GrpE protein homolog, mitochondrial; 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. Seems to control the nucleotide-dependent binding of ssc1 to substrate proteins (By similarity). (223 aa) |
| | ndi1 | Probable NADH-ubiquinone oxidoreductase C947.15c, mitochondrial; Catalyzes the oxidation of NADH. (551 aa) |
| | oxa102 | Mitochondrial inner membrane protein oxa1-2; Required for the insertion of integral membrane proteins into the mitochondrial inner membrane. Essential for the activity and assembly of cytochrome c oxidase. It is essential for viability while oxa101 is not. When both are deleted the cell is non-viable, suggesting that oxa101 act as a back-up for oxa102; Belongs to the OXA1/ALB3/YidC family. (409 aa) |
| | atp14 | ATP synthase subunit H, 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 [...] (103 aa) |
| | rsm23 | 37S ribosomal protein S23, mitochondrial; Required for maintenance of mitochondrial DNA. (476 aa) |
| | cyt1 | Cytochrome c1, heme protein, 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 gradient ove [...] (307 aa) |
| | mti2 | Translation initiation factor IF-2, mitochondrial; One of the essential components for the initiation of protein synthesis. Protects formylmethionyl-tRNA from spontaneous hydrolysis and promotes its binding to the 30S ribosomal subunits. Also involved in the hydrolysis of GTP during the formation of the 70S ribosomal complex (By similarity); Belongs to the TRAFAC class translation factor GTPase superfamily. Classic translation factor GTPase family. IF-2 subfamily. (686 aa) |
| | cys11 | Cysteine synthase 1; Catalyzes the conversion of O-succinyl-L-serine into cysteine, the last step in the cysteine biosynthesis pathway. Can also use O-acetyl-L-serine ; Belongs to the cysteine synthase/cystathionine beta- synthase family. (351 aa) |
| | arh1 | Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Serves as the first electron transfer protein in all the mitochondrial P450 systems. (469 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). (550 aa) |
| | hem15 | Ferrochelatase, mitochondrial; Catalyzes the ferrous insertion into protoporphyrin IX. Belongs to the ferrochelatase family. (423 aa) |
| | lat1 | Pyruvate dehydrogenase E2 component (dihydrolipoamide acetyltransferase); The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). (483 aa) |
| | nam9 | 37S ribosomal protein S4-like, mitochondrial. (327 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. Recruits mitochondrial HSP70 and its co-chaperone (mge1) to drive protein translocation into the matrix using ATP as an energy source. (427 aa) |
| | mrpl24 | 54S ribosomal protein L24, mitochondrial. (176 aa) |
| | bot1 | 37S ribosomal protein S35, mitochondrial; Required for mitochondrial protein synthesis. Has a role in mitochondrial integrity and cell respiration. (315 aa) |
| | SPBC15C4.02 | ABC1 family protein MCP2 homolog; Involved in mitochondrial lipid homeostasis. Belongs to the protein kinase superfamily. ADCK protein kinase family. (557 aa) |
| | mrpl23 | 54S ribosomal protein L23, mitochondrial; Belongs to the universal ribosomal protein uL13 family. (157 aa) |
| | ppr2 | Pentatricopeptide repeat-containing protein 2, mitochondrial; Mitochondrial RNA-binding protein that acts as a general translation factor. (432 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). (228 aa) |
| | mug98 | Meiotically up-regulated gene 98 protein, mitochondrial; Has a role in meiosis. (113 aa) |
| | tam41 | Phosphatidate cytidylyltransferase, mitochondrial; Catalyzes the formation of CDP-diacylglycerol (CDP-DAG) from phosphatidic acid (PA) in the mitochondrial inner membrane. Required for the biosynthesis of the dimeric phospholipid cardiolipin, which stabilizes supercomplexes of the mitochondrial respiratory chain in the mitochondrial inner membrane. (393 aa) |
| | nfs1 | Probable 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. Belongs to the class-V pyridoxal-phosphate-dependent aminotransferase family. NifS/IscS subfamily. (501 aa) |
| | kgd1 | 2-oxoglutarate dehydrogenase, mitochondrial; The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3) (By similarity). (1009 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. (803 aa) |
| | coq3 | Ubiquinone biosynthesis O-methyltransferase, mitochondrial; O-methyltransferase that catalyzes the 2 O-methylation steps in the ubiquinone biosynthetic pathway. (274 aa) |
| | mrpl22 | 54S ribosomal protein L22, mitochondrial. (249 aa) |
| | cox13 | Cytochrome c oxidase subunit 13, mitochondrial; 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 [...] (130 aa) |
| | atp5 | ATP synthase subunit 5, 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 [...] (216 aa) |
| | inh1 | Putative ATPase inhibitor, mitochondrial; Forms a one-to-one complex with ATPase to inhibit the enzyme activity completely. (90 aa) |
| | put1 | Probable proline dehydrogenase, mitochondrial; Converts proline to delta-1-pyrroline-5-carboxylate. Belongs to the proline oxidase family. (492 aa) |
| | arg1 | Probable acetylornithine aminotransferase, mitochondrial; Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family. (441 aa) |
| | rrf1 | Putative ribosomal recycling factor, mitochondrial; Necessary for protein synthesis in mitochondria. Functions as a ribosome recycling factor in mitochondria; Belongs to the RRF family. (244 aa) |
| | atp3 | ATP synthase subunit gamma, 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 [...] (301 aa) |
| | SPBC24C6.03 | Probable proline--tRNA ligase, mitochondrial; Belongs to the class-II aminoacyl-tRNA synthetase family. (425 aa) |
| | mtg1 | Mitochondrial GTPase 1; Mitochondrial GTPase involved in assembly of the large ribosomal subunit (By similarity). Plays a role in expression of the mitochondrial translational machinery (By similarity). Belongs to the TRAFAC class YlqF/YawG GTPase family. MTG1 subfamily. (328 aa) |
| | snr1 | 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has an indirect role in endocytic membrane trafficking. May have a function in protein biosynthesis in mitochondrial small ribosomal subunit (By similarity). (429 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. (216 aa) |
| | fxn1 | Frataxin homolog, mitochondrial; Promotes the biosynthesis of heme as well as the assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+). May be able to store large amounts of the metal in the form of a ferrihydrite mineral by oligomerization (By similarity); Belongs to the frataxin family. (158 aa) |
| | pet127 | mRNA degradation protein pet127, mitochondrial; Required for the degradation of mRNA in mitochondria. Belongs to the PET127 family. (524 aa) |
| | msk1 | Lysine--tRNA ligase, mitochondrial; Belongs to the class-II aminoacyl-tRNA synthetase family. (531 aa) |
| | sdh3 | Succinate dehydrogenase cytochrome B subunit, mitochondrial; Membrane-anchoring 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). (180 aa) |
| | SPCC576.06c | Tyrosine--tRNA ligase, mitochondrial; Catalyzes the attachment of tyrosine to tRNA(Tyr) in a two- step reaction: tyrosine is first activated by ATP to form Tyr-AMP and then transferred to the acceptor end of tRNA(Tyr). Belongs to the class-I aminoacyl-tRNA synthetase family. (445 aa) |
| | msf1 | Phenylalanine--tRNA ligase, mitochondrial; Is responsible for the charging of tRNA(Phe) with phenylalanine in mitochondrial translation. (429 aa) |
| | mrps8 | 37S ribosomal protein subunit S8, mitochondrial; Belongs to the universal ribosomal protein uS8 family. (152 aa) |
| | fyv4 | Protein fyv4, mitochondrial; Involved in telomere length regulation; Belongs to the mitochondrion-specific ribosomal protein mS41 family. (113 aa) |
| | cox5 | Cytochrome c oxidase polypeptide 5, mitochondrial; 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 transmembr [...] (186 aa) |
| | dar2 | Aspartate--tRNA ligase, mitochondrial; Belongs to the class-II aminoacyl-tRNA synthetase family. Type 1 subfamily. (611 aa) |
| | hmt2 | Sulfide:quinone oxidoreductase, mitochondrial; Catalyzes the oxidation of hydrogen sulfide, with the help of a quinone. (459 aa) |
| | cem1 | Putative 3-oxoacyl-[acyl-carrier-protein] synthase, mitochondrial; May play a role in the biosynthesis of lipoic acid as well as longer chain fatty acids required for optimal mitochondrial function. Belongs to the thiolase-like superfamily. Beta-ketoacyl-ACP synthases family. (426 aa) |
| | maa1 | Aspartate aminotransferase, mitochondrial; Plays a key role in amino acid metabolism. Important for metabolite exchange between mitochondria and cytosol (By similarity). Belongs to the class-I pyridoxal-phosphate-dependent aminotransferase family. (437 aa) |
| | arg6 | Amino-acid acetyltransferase, mitochondrial; N-acetylglutamate synthase involved in arginine biosynthesis. Belongs to the acetyltransferase family. (500 aa) |
| | ppr1 | Pentatricopeptide repeat-containing protein 1, mitochondrial; Mitochondrial RNA-binding protein required for the stability of the cox2 and cox3 mRNAs. (697 aa) |
| | atp4 | ATP synthase subunit 4, 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 [...] (244 aa) |
| | atp17 | ATP synthase subunit f, 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 [...] (96 aa) |
| | mrpl32 | Probable 54S ribosomal protein L32, mitochondrial; Component of the large subunit of mitochondrial ribosome. Belongs to the bacterial ribosomal protein bL32 family. (103 aa) |
| | mrpl17 | 54S ribosomal protein L17, mitochondrial; Belongs to the mitochondrion-specific ribosomal protein mL46 family. (268 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. (433 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. (813 aa) |
| | rpm2 | 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). (647 aa) |
| | SPBC18E5.10 | NADH-ubiquinone oxidoreductase 51 kDa subunit homolog SPBC18E5.10, mitochondrial. (452 aa) |
| | trx2 | Thioredoxin-2, mitochondrial; Disulfide reductase which serves multiple functions in mitochondria, protecting mitochondrial components against thiol- oxidative damage as a thiol-disulfide oxidoreductase, and supporting urea cycle and respiration in mitochondria in a manner independent of active site thiols. (133 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. (209 aa) |
| | fum1 | Fumarate hydratase, mitochondrial; Catalyzes the reversible stereospecific interconversion of fumarate to L-malate. In mitochondrion, catalyzes the hydration of fumarate to L-malate in the tricarboxylic acid (TCA) cycle to facilitate a transition step in the production of energy in the form of NADH. In cytoplasm and nucleus, involved in DNA repair in response to DNA damage: following DNA double-strand breaks (DSBs), translocates from the cytosol to the nucleus and promotes DNA repair by catalyzing the dehydration of L-malate to fumarate. Belongs to the class-II fumarase/aspartase famil [...] (520 aa) |
| | cox18 | Cytochrome c oxidase assembly protein cox18, mitochondrial; Required for the insertion of integral membrane proteins into the mitochondrial inner membrane. Essential for the activity and assembly of cytochrome c oxidase. Plays a central role in the translocation and export of the C-terminal part of the cox2 protein into the mitochondrial intermembrane space. Belongs to the OXA1/ALB3/YidC family. (202 aa) |
| | ppr8 | Pentatricopeptide repeat-containing protein 8, mitochondrial; Mitochondrial RNA-binding protein involved in mitochondrial translation. The cox1 mRNA is one target but it is not clear if ppr8 has a single or multiple targets. (481 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). (223 aa) |
| | mrm1 | rRNA methyltransferase 1, mitochondrial; S-adenosyl-L-methionine-dependent 2'-O-ribose methyltransferase that catalyzes the formation of the 2'-O- methylguanosine corresponding to position 2270 in S.cerevisiae 21S mitochondrial large subunit ribosomal RNA (mtLSU rRNA), a universally conserved modification in the peptidyl transferase domain of the mtLSU rRNA. (301 aa) |
| | tda1 | Threonine dehydratase, mitochondrial; Belongs to the serine/threonine dehydratase family. (600 aa) |
| | ppk24 | Serine/threonine-protein kinase ppk24, mitochondrial; Has a role late in meiosis; Belongs to the protein kinase superfamily. Ser/Thr protein kinase family. (461 aa) |
| | mam33 | Mitochondrial acidic protein mam33. (269 aa) |
| | kgd2 | 2-oxoglutarate dehydrogenase E2 component (dihydrolipoamide succinyltransferase); The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3) (By similarity). (452 aa) |
| | SPBC83.05 | 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. (773 aa) |
| | rtc6 | 54S ribosomal protein c83.06c, mitochondrial; Belongs to the bacterial ribosomal protein bL36 family. (92 aa) |
| | pdx1 | Probable pyruvate dehydrogenase protein X component, mitochondrial; Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex (By similarity). (456 aa) |
| | pgp1 | 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. (412 aa) |
| | mrpl20 | 54S ribosomal protein L20, mitochondrial. (197 aa) |
| | mas2 | Probable mitochondrial-processing peptidase subunit alpha; Substrate recognition and binding subunit of the essential mitochondrial processing protease (MPP), which cleaves the mitochondrial sequence off newly imported precursors proteins. (502 aa) |
| | dpc13 | Uncharacterized protein PB1A10.16, mitochondrial. (143 aa) |
| | ina17 | Inner membrane assembly complex subunit 17; Component of the INA complex (INAC) that promotes the biogenesis of mitochondrial F(1)F(0)-ATP synthase. INAC facilitates the assembly of the peripheral stalk and promotes the assembly of the catalytic F(1)-domain with the membrane-embedded F(0)-domain. (112 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). (275 aa) |
| | atp2 | ATP synthase subunit beta, 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 c [...] (525 aa) |
| | ssc1 | Heat shock 70 kDa protein, mitochondrial; Belongs to the heat shock protein 70 family. (674 aa) |
| | atp1 | ATP synthase subunit alpha, 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 [...] (536 aa) |
| | arg3 | Ornithine carbamoyltransferase, mitochondrial; Ornithine carbamoyltransferase involved in the synthesis of arginine from glutamate via ornithine and the urea cycle. (327 aa) |
| | arg11 | N-acetyl-gamma-glutamyl-phosphate reductase; In the C-terminal section; belongs to the NAGSA dehydrogenase family. (885 aa) |
| | ura3 | 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; Belongs to the dihydroorotate dehydrogenase family. Type 2 subfamily. (443 aa) |
| | ilv1 | Acetolactate synthase, mitochondrial; Belongs to the TPP enzyme family. (669 aa) |
| | mrpl44 | 54S ribosomal protein L44, mitochondrial; Involved in mitochondrial genome encoded proteins translation. (100 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 [...] (426 aa) |
| | etf1 | Probable electron transfer flavoprotein subunit alpha, mitochondrial; The electron transfer flavoprotein serves as a specific electron acceptor for several dehydrogenases, including five acyl-CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase. It transfers the electrons to the main mitochondrial respiratory chain via ETF- ubiquinone oxidoreductase (ETF dehydrogenase) (By similarity). (341 aa) |
| | ilv5 | Probable ketol-acid reductoisomerase, mitochondrial; Belongs to the ketol-acid reductoisomerase family. (404 aa) |
| | mrm2 | rRNA methyltransferase 2, mitochondrial; S-adenosyl-L-methionine-dependent 2'-O-ribose methyltransferase that catalyzes the formation of the 2'-O- methyluridine corresponding to position 2791 in S.cerevisiae 21S mitochondrial large subunit ribosomal RNA (mtLSU rRNA), a universally conserved modification in the peptidyl transferase domain of the mtLSU rRNA. (218 aa) |
| | cox4 | Cytochrome c oxidase subunit 4, mitochondrial; 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 [...] (164 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 [...] (313 aa) |
| | SPAC20G8.02 | Probable phospholipase C20G8.02, mitochondrial; Probable phospholipase that hydrolyzes phosphatidic acid. Belongs to the PA-PLA1 family. (757 aa) |
| | SPAC20G8.04c | Probable electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial; Accepts electrons from ETF and reduces ubiquinone. (632 aa) |
| | atp11 | Protein atp11, mitochondrial; Essential for the assembly of the mitochondrial F1-F0 complex. May interact with the alpha and/or beta subunits of F1-ATPase (By similarity); Belongs to the ATP11 family. (286 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. (422 aa) |
| | trz2 | Ribonuclease Z 2, mitochondrial; Zinc phosphodiesterase, which displays some tRNA 3'- processing endonuclease activity. May be involved in tRNA maturation, by removing a 3'-trailer from precursor tRNA. Belongs to the RNase Z family. (678 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). (305 aa) |
| | mrpl4 | 54S ribosomal protein L4, mitochondrial; Belongs to the universal ribosomal protein uL29 family. (144 aa) |
| | cue1 | CUE domain-containing protein 4, mitochondrial. (215 aa) |
| | mdj1 | DnaJ homolog 1, mitochondrial. (528 aa) |
| | msp1 | Protein msp1, mitochondrial; Dynamin-related GTPase required for mitochondrial fusion. Coordinates interaction between the inner and outer mitochondrial membrane to promote the formation of the double membrane (By similarity). (903 aa) |
| | rip1 | Cytochrome b-c1 complex subunit Rieske, 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 g [...] (228 aa) |
| | pdb1 | Pyruvate dehydrogenase E1 component subunit beta, mitochondrial; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). (366 aa) |
| | adh4 | Alcohol dehydrogenase 4; Involved in ethanol oxidation in mitochondria. Belongs to the iron-containing alcohol dehydrogenase family. (422 aa) |
| | mrf1 | Putative peptide chain release factor 1, mitochondrial; Belongs to the prokaryotic/mitochondrial release factor family. (396 aa) |
| | rsm24 | 37S ribosomal protein S24, mitochondrial; Involved in mitochondrial genome encoded proteins translation. (258 aa) |
| | mrp11 | 54S ribosomal protein L11, mitochondrial; Involved in mitochondrial genome encoded proteins translation. (216 aa) |
| | atp25 | ATPase synthesis protein 25, mitochondrial; mRNA stabilization factor specific for the 0.95 kb oli1 mRNA. Also involved in oli1 ring formation (By similarity); Belongs to the ATP25 family. (542 aa) |
| | mrp20 | 54S ribosomal protein L23, mitochondrial; Involved in mitochondrial genome encoded proteins translation. (161 aa) |
| | lon1 | 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. (1067 aa) |
| | gcv2 | Putative glycine dehydrogenase (decarboxylating), mitochondrial; The glycine cleavage system catalyzes the degradation of glycine. The P protein binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor; CO(2) is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein; Belongs to the GcvP family. (1031 aa) |
| | SPAC4G8.09 | Putative leucine--tRNA ligase, mitochondrial; Belongs to the class-I aminoacyl-tRNA synthetase family. (874 aa) |
| | mcp60 | 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; Belongs to the chaperonin (HSP60) family. (582 aa) |
| | mrpl6 | Putative 54S ribosomal protein L6, mitochondrial. (213 aa) |
| | iba57 | Putative transferase caf17, mitochondrial. (325 aa) |
| | mdm31 | Mitochondrial distribution and morphology protein 31; Involved in the organization of the mitochondrial membranes and the global structure of the mitochondria. Also required for mitochondrial distribution and mobility as well as for the maintenance of mitochondrial DNA nucleoids structures (By similarity). Belongs to the MDM31/MDM32 family. (601 aa) |
| | SPAC3H1.08c | Uncharacterized CCDC90 family protein C3H1.08c, mitochondrial. (211 aa) |
| | shm2 | Serine hydroxymethyltransferase, mitochondrial; Interconversion of serine and glycine; Belongs to the SHMT family. (488 aa) |
| | img2 | 54S ribosomal protein img2, mitochondrial; Belongs to the mitochondrion-specific ribosomal protein mL49 family. (105 aa) |
| | rdl2 | Putative thiosulfate sulfurtransferase, mitochondrial; Thiosulfate sulfurtransferase which catalyzes the transfer of sulfane sulfur from thiosulfate to cyanide. (142 aa) |
| | SPAC4H3.09 | Putative acyl carrier protein, mitochondrial; Carrier of the growing fatty acid chain in fatty acid biosynthesis (By similarity). May be involved in the synthesis of very- long-chain fatty acids; Belongs to the acyl carrier protein (ACP) family. (112 aa) |
| | cit1 | Probable citrate synthase, mitochondrial; Belongs to the citrate synthase family. (483 aa) |
| | ilv3 | Putative dihydroxy-acid dehydratase, mitochondrial. (598 aa) |
| | cys2 | Serine O-succinyltransferase; Transfers a succinyl group from succinyl-CoA to L-serine, forming succinyl-L-serine. Has also weak serine acetyl transferase activity and homoserine succinyl transferase activity. (504 aa) |
| | etp1 | Electron transfer protein 1, mitochondrial. (616 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). (762 aa) |
| | cce1 | Cruciform cutting endonuclease 1, mitochondrial; Capable of resolving Holliday junctions. Specific for 4-way junctions. Seems to be important for the maintenance of mitochondrial DNA. Cleaves fixed junctions at the point of strand exchange. Cleaves after 5'-CT-3' and 5'-TT-3' sequences. (258 aa) |
| | icp55 | Intermediate cleaving peptidase 55; Aminopeptidase which cleaves preprotein intermediates that carry destabilizing N-ter amino acid residues after the mitochondrial processing peptidase (MPP) cleavage site and is thus critical for stabilization of the mitochondrial proteome. (486 aa) |
| | emi5 | 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. (139 aa) |
| | ppr5 | Pentatricopeptide repeat-containing protein 5, mitochondrial; Mitochondrial RNA-binding protein that acts as a general negative regulator of mitochondrial translation. (1261 aa) |
| | pnu1 | Nuclease 1, mitochondrial; This enzyme has both RNase and DNase activity. It degrades single-stranded DNA and RNA. (322 aa) |
| | etr1 | Enoyl-[acyl-carrier-protein] reductase, mitochondrial; Catalyzes the NADPH-dependent reduction of trans-2-enoyl thioesters in mitochondrial fatty acid synthesis (fatty acid synthesis type II). Fatty acid chain elongation in mitochondria uses acyl carrier protein (ACP) as an acyl group carrier, but the enzyme accepts both ACP and CoA thioesters as substrates in vitro. Required for respiration and the maintenance of the mitochondrial compartment. Belongs to the zinc-containing alcohol dehydrogenase family. Quinone oxidoreductase subfamily. (372 aa) |
| | pda1 | Pyruvate dehydrogenase E1 component subunit alpha, mitochondrial; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). (409 aa) |
| | rpm1 | Exoribonuclease II, mitochondrial; Required for intron-independent turnover and processing of mitochondrial RNA. Participates in 3'-mtRNA processing where it hydrolyzes single-stranded RNA or partially double-stranded RNA with 3'-single-stranded tails; Belongs to the RNR ribonuclease family. (957 aa) |
| | mrpl27 | 54S ribosomal protein L27, mitochondrial; Belongs to the mitochondrion-specific ribosomal protein mL41 family. (93 aa) |
| | mti3 | Probable translation initiation factor, mitochondrial; May be involved in mitochondrial translation initiation. Belongs to the IF-3 family. (233 aa) |
| | cox1102 | Cytochrome c oxidase assembly protein cox11-2; Rsm22-2 is involved in mitochondrion-encoded protein synthesis; In the C-terminal section; belongs to the COX11/CtaG family. (753 aa) |
| | coq8 | Protein ABC1 homolog, mitochondrial. (610 aa) |
| | dld2 | Putative D-lactate dehydrogenase C713.03, mitochondrial. (526 aa) |
| | mrp7 | 54S ribosomal protein L27, mitochondrial; Involved in mitochondrial genome encoded proteins translation. (153 aa) |
| | gcv3 | Putative glycine cleavage system H protein, mitochondrial; The glycine cleavage system (glycine decarboxylase complex) catalyzes the degradation of glycine. The H protein shuttles the methylamine group of glycine from the P protein to the T protein (By similarity); Belongs to the GcvH family. (169 aa) |
| | mse1 | Glutamate--tRNA ligase, mitochondrial; Catalyzes the attachment of glutamate to tRNA(Glu) in a two- step reaction: glutamate is first activated by ATP to form Glu-AMP and then transferred to the acceptor end of tRNA(Glu). (526 aa) |
| | csr101 | CRAL-TRIO domain-containing protein C589.09, mitochondrial. (388 aa) |
| | tsf1 | Elongation factor Ts, mitochondrial; Associates with the EF-Tu.GDP complex and induces the exchange of GDP to GTP. It remains bound to the aminoacyl-tRNA.EF- Tu.GTP complex up to the GTP hydrolysis stage on the ribosome. Belongs to the EF-Ts family. (299 aa) |
| | ght8 | Probable high-affinity hexose transporter ght8, mitochondrial. (547 aa) |
| | cox8 | Cytochrome c oxidase polypeptide VIII, mitochondrial; 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 transme [...] (66 aa) |
| | mug99 | Meiotically up-regulated gene 99 protein, mitochondrial; Required for correct meiotic chromosome segregation. Appears to also have role in sporulation. (526 aa) |
| | mrpl1 | 54S ribosomal protein L1, mitochondrial; Belongs to the universal ribosomal protein uL1 family. (253 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). (317 aa) |
| | mrpl9 | 54S ribosomal protein L9, mitochondrial; Belongs to the universal ribosomal protein uL3 family. (259 aa) |
| | pkp1 | [Pyruvate dehydrogenase (acetyl-transferring)] kinase, mitochondrial; Inhibits the mitochondrial pyruvate dehydrogenase complex by phosphorylation of the E1 alpha subunit, thus contributing to the regulation of glucose metabolism. (425 aa) |
| | atp16 | ATP synthase subunit delta, 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 turnover in the catalytic domain of F(1) is coupled via a rotary mechanism of the c [...] (167 aa) |
| | aco2 | Homocitrate dehydratase, mitochondrial; Catalyzes the reversible dehydration of (R)-homocitrate to cis-homoaconitate, a step in the alpha-aminoadipate pathway for lysine biosynthesis. (918 aa) |
| | rbd3 | Uncharacterized rhomboid protein P4H10.10, mitochondrial. (392 aa) |
| | mrx14 | Probable 54S ribosomal protein L34, mitochondrial. (108 aa) |
| | SPBC21C3.03 | ABC1 family protein C21C3.03, mitochondrial. (692 aa) |
| | mgr3 | TPR repeat-containing protein P27G11.02. (356 aa) |
| | tim18 | Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial; Membrane-anchoring 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). (159 aa) |
| | qcr1 | Probable mitochondrial-processing peptidase subunit beta; Catalytic subunit of the essential mitochondrial processing protease (MPP), which cleaves the mitochondrial sequence off newly imported precursors proteins (By similarity). Preferentially, cleaves after an arginine at position P2 (By similarity). Belongs to the peptidase M16 family. (457 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 sdh2 of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants. May act together with SDHAF1. (115 aa) |
| | SPCC777.17c | Probable 54S ribosomal protein L9, mitochondrial. (101 aa) |
| | sod2 | Superoxide dismutase [Mn], mitochondrial; Destroys superoxide anion radicals which are normally produced within the cells and which are toxic to biological systems; Belongs to the iron/manganese superoxide dismutase family. (218 aa) |
| | mmf2 | Protein mmf2, mitochondrial; Plays a role in the maintenance of mitochondrial DNA. Belongs to the RutC family. (126 aa) |
| | mrpl31 | 54S ribosomal protein L31, mitochondrial; Belongs to the mitochondrion-specific ribosomal protein mL60 family. (115 aa) |
| | SPCP31B10.02 | UPF0651 protein P31B10.02, mitochondrial. (143 aa) |
| | mrpl12 | 54S ribosomal protein L12, mitochondrial; Belongs to the bacterial ribosomal protein bL12 family. (173 aa) |
| | rsm18 | 37S ribosomal protein rsm18, mitochondrial; Belongs to the bacterial ribosomal protein bS18 family. (166 aa) |
| | ppr6 | Pentatricopeptide repeat-containing protein 6, mitochondrial; Mitochondrial RNA-binding protein required for the stability of the atp9 mRNA. (443 aa) |
| | idh2 | Isocitrate dehydrogenase [NAD] subunit 2, mitochondrial; Performs an essential role in the oxidative function of the citric acid cycle and is involved in glutamate biosynthesis. Also binds RNA; specifically to the 5'-untranslated leaders of mitochondrial mRNAs. (379 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. (770 aa) |
| | sls1 | Protein sls1; Involved in aerobic respiration where it is required for assembly of respiratory chain enzyme complexes III and IV. Also has a role in mitochondrial gene expression (By similarity); Belongs to the SLS1 family. (677 aa) |
| | mtg2 | Uncharacterized GTP-binding protein P8A3.11c, mitochondrial. (419 aa) |
| | atp12 | Protein atp12, mitochondrial; Essential for the assembly of the mitochondrial F1-F0 complex; Belongs to the ATP12 family. (291 aa) |
| | lys2 | Homoaconitase, mitochondrial; Catalyzes the reversible hydration of cis-homoaconitate to (2R,3S)-homoisocitrate, a step in the alpha-aminoadipate pathway for lysine biosynthesis. (721 aa) |
| | pos5 | NADH kinase pos5, mitochondrial; Phosphorylates both NADH and NAD(+), with a preference for NADH. Anti-oxidant factor and key source of the cellular reductant NADPH (By similarity). (386 aa) |
| | SPAC27E2.11c | Uncharacterized protein C27E2.11c, mitochondrial. (81 aa) |
| | SPAC25B8.18 | Putative redox protein fmp46, mitochondrial; Putative mitochondrial redox protein which could be involved in the reduction of small toxic molecules; Belongs to the FMP46 family. (121 aa) |
| | dia4 | Serine--tRNA ligase, mitochondrial; Catalyzes the attachment of serine to tRNA(Ser). Is also probably able to aminoacylate tRNA(Sec) with serine, to form the misacylated tRNA L-seryl-tRNA(Sec), which will be further converted into selenocysteinyl-tRNA(Sec) (By similarity). (479 aa) |
| | psd2 | Phosphatidylserine decarboxylase proenzyme 2, 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). Together with psd1 and psd3, responsible for the majority of phosphatidylethanolamine synthesis. Belongs to the phosphatidylserine decarboxylase family. PSD-B subfamily. Eukaryotic type I sub-subfamily. (516 aa) |
| | isu1 | Iron sulfur cluster assembly protein 1, mitochondrial; 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. First, a [2Fe-2S] cluster is transiently assembled on the scaffold protein isu1. In a second step, the cluster is released from isu1, transferred to a glutaredoxin, followed by the formation of mitochondrial [2Fe-2S] proteins, the synthesis of [4Fe-4S] clusters and their target-specific insertion into the recipient apoproteins. Cluster a [...] (192 aa) |
| | mss1 | tRNA modification GTPase mss1, mitochondrial; GTPase involved in the 5-carboxymethylaminomethyl modification (mnm(5)s(2)U34) of the wobble uridine base in mitochondrial tRNAs; Belongs to the TRAFAC class TrmE-Era-EngA-EngB-Septin-like GTPase superfamily. TrmE GTPase family. (496 aa) |
| | cox6 | Cytochrome c oxidase subunit 6, mitochondrial; 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 [...] (140 aa) |
| | sdh1 | Probable succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial; Flavoprotein (FP) 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); Belongs to the FAD-dependent oxidoreductase 2 family. FRD/SDH subfamily. (641 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. (113 aa) |
| | mrpl19 | 54S ribosomal protein L19, mitochondrial; Belongs to the universal ribosomal protein uL11 family. (144 aa) |
| | jac1 | J-type co-chaperone jac1, mitochondrial; Co-chaperone required for the assembly of iron-sulfur (Fe/S) clusters in mitochondria. Stimulates the ATPase activity of the mitochondrial Hsp70 chaperone (mtHsp70). Binds to the substrate protein isu1 and targets it to mtHsp70 (By similarity); Belongs to the HscB family. (225 aa) |
| | cox1101 | Cytochrome c oxidase assembly protein cox11-1; Rsm22-1 is involved in mitochondrion-encoded protein synthesis; In the C-terminal section; belongs to the COX11/CtaG family. (753 aa) |
| | pfh1 | ATP-dependent DNA helicase pfh1; DNA-dependent ATPase and 5'-3' DNA helicase required for the maintenance of both mitochondrial and nuclear genome stability. Involved in the maintenance of mitochondrial (mtDNA). Required for both repair of mitochondrial DNA and recognition of a recombinogenic signal characterized by a 26-bp palindromic at sequence in the ery region of mitochondrial DNA. May have a general role in chromosomal replication by affecting Okazaki fragment maturation. Required for the completion of S-phase. Belongs to the helicase family. PIF1 subfamily. (805 aa) |
| | mrpl25 | 54S ribosomal protein L25, mitochondrial; Belongs to the mitochondrion-specific ribosomal protein mL59 family. (145 aa) |
| | tuf1 | Elongation factor Tu, mitochondrial; This protein promotes the GTP-dependent binding of aminoacyl- tRNA to the A-site of ribosomes during protein biosynthesis. Belongs to the TRAFAC class translation factor GTPase superfamily. Classic translation factor GTPase family. EF-Tu/EF-1A subfamily. (439 aa) |
| | mrpl10 | 54S ribosomal protein L10, mitochondrial; Belongs to the universal ribosomal protein uL15 family. (254 aa) |
| | mdl1 | ATP-dependent permease MDL1, mitochondrial; Mediates export of peptides generated upon proteolysis of mitochondrial inner membrane proteins; Belongs to the ABC transporter superfamily. ABCB family. Mitochondrial peptide exporter (TC 3.A.1.212) subfamily. (726 aa) |
| | mdh1 | Malate dehydrogenase, mitochondrial. (341 aa) |
| | mrpl37 | 54S ribosomal protein L37, mitochondrial; Belongs to the mitochondrion-specific ribosomal protein mL54 family. (139 aa) |
| | cox10 | Protoheme IX farnesyltransferase, mitochondrial; Converts protoheme IX and farnesyl diphosphate to heme O. (387 aa) |
| | mrp15 | 54S ribosomal protein L15, mitochondrial; Belongs to the ribonuclease III family. Mitochondrion- specific ribosomal protein mL57 subfamily. (210 aa) |
| | pth3 | Uncharacterized peptide chain release factor-like protein C1105.18c, mitochondrial. (162 aa) |
| | mrpl16 | 54S ribosomal protein L16, mitochondrial. (215 aa) |
| | lys4 | Homocitrate synthase, mitochondrial; Catalyzes the aldol-type condensation of 2-oxoglutarate with acetyl-CoA to yield homocitrate. Carries out the first step of the alpha-aminoadipate (AAA) lysine biosynthesis pathway. (418 aa) |
