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| | PUT2 | Delta-1-pyrroline-5-carboxylate dehydrogenase; nuclear-encoded mitochondrial protein involved in utilization of proline as sole nitrogen source; deficiency of human homolog ALDH4A1 causes type II hyperprolinemia (HPII), an autosomal recessive inborn error of metabolism; human homolog ALDH4A1 can complement yeast null mutant. (575 aa) |
| | FYV4 | Protein FYV4, mitochondrial; Protein of unknown function; required for survival upon exposure to K1 killer toxin; Belongs to the mitochondrion-specific ribosomal protein mS41 family. (130 aa) |
| | TRM5 | tRNA(m(1)G37)methyltransferase; methylates a tRNA base adjacent to the anticodon that has a role in prevention of frameshifting; localized to both cytoplasm and mitochondria, and modifies both cytoplasmic and mitochondrial tRNAs; mutations in human ortholog TRMT5 are associated with skeletal muscle respiratory chain deficiencies, and trm5 mutations analogous to disease mutations decrease respiration; Belongs to the class I-like SAM-binding methyltransferase superfamily. TRM5/TYW2 family. (499 aa) |
| | PPE1 | Protein phosphatase methylesterase 1; Protein with carboxyl methyl esterase activity; may have a role in demethylation of the phosphoprotein phosphatase catalytic subunit; also identified as a small subunit mitochondrial ribosomal protein; Belongs to the AB hydrolase superfamily. (400 aa) |
| | GEP4 | Mitochondrial phosphatidylglycerophosphatase (PGP phosphatase); dephosphorylates phosphatidylglycerolphosphate to generate phosphatidylglycerol, an essential step during cardiolipin biosynthesis; null mutant is sensitive to tunicamycin, DTT; Belongs to the GEP4 family. (185 aa) |
| | MRPL6 | Mitochondrial ribosomal protein of the large subunit. (214 aa) |
| | BAT1 | Branched-chain-amino-acid aminotransferase, mitochondrial; Mitochondrial branched-chain amino acid (BCAA) aminotransferase; preferentially involved in BCAA biosynthesis; homolog of murine ECA39; highly expressed during logarithmic phase and repressed during stationary phase; BAT1 has a paralog, BAT2, that arose from the whole genome duplication; Belongs to the class-IV pyridoxal-phosphate-dependent aminotransferase family. (393 aa) |
| | TIM44 | Mitochondrial import inner membrane translocase subunit TIM44; Essential component of the TIM23 complex; tethers the import motor and regulatory factors (PAM complex) to the translocation channel (Tim23p-Tim17p core complex); TIM23 complex is short for the translocase of the inner mitochondrial membrane; Belongs to the Tim44 family. (431 aa) |
| | MMF1 | Mitochondrial protein required for transamination of isoleucine; but not of valine or leucine; may regulate specificity of branched-chain transaminases Bat1p and Bat2p; induction of expression in response to stress is mediated by a Hog1p-regulated antisense RNA and gene looping; interacts genetically with mitochondrial ribosomal protein genes; MMF1 has a paralog, HMF1, that arose from the whole genome duplication. (145 aa) |
| | MAM33 | Mitochondrial acidic protein MAM33; Specific translational activator for the mitochondrial COX1 mRNA; acidic protein of the mitochondrial matrix; related to the human complement receptor gC1q-R; Belongs to the MAM33 family. (266 aa) |
| | RSM25 | Mitochondrial ribosomal protein of the small subunit. (264 aa) |
| | FMC1 | ATP synthase assembly factor FMC1, mitochondrial; Mitochondrial matrix protein; required for assembly or stability at high temperature of the F1 sector of mitochondrial F1F0 ATP synthase; null mutant temperature sensitive growth on glycerol is suppressed by multicopy expression of Odc1p. (155 aa) |
| | KGD1 | 2-oxoglutarate dehydrogenase, mitochondrial; Subunit of the mitochondrial alpha-ketoglutarate dehydrogenase complex; catalyzes a key step in the tricarboxylic acid (TCA) cycle, the oxidative decarboxylation of alpha-ketoglutarate to form succinyl-CoA. (1014 aa) |
| | MRPL8 | Mitochondrial ribosomal protein of the large subunit. (238 aa) |
| | ARG2 | Amino-acid acetyltransferase, mitochondrial; Acetylglutamate synthase (glutamate N-acetyltransferase); mitochondrial enzyme that catalyzes the first step in the biosynthesis of the arginine precursor ornithine; forms a complex with Arg5,6p. (574 aa) |
| | MRPL49 | Mitochondrial ribosomal protein of the large subunit. (161 aa) |
| | PAM16 | Subunit of the import motor (PAM complex); the PAM complex is a component of the Translocase of the Inner Mitochondrial membrane (TIM23 complex); forms a 1:1 subcomplex with Pam18p and inhibits its cochaperone activity; contains a J-like domain; Belongs to the TIM16/PAM16 family. (149 aa) |
| | SSC1 | Heat shock protein SSC1, mitochondrial; Hsp70 family ATPase; constituent of the import motor component of the Translocase of the Inner Mitochondrial membrane (TIM23 complex); involved in protein translocation and folding; subunit of SceI endonuclease; SSC1 has a paralog, ECM10, that arose from the whole genome duplication. (654 aa) |
| | RSM26 | Mitochondrial ribosomal protein of the small subunit. (266 aa) |
| | RSM7 | Mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S7 ribosomal protein. (247 aa) |
| | IBA57 | Putative transferase CAF17, mitochondrial; Protein involved in incorporating iron-sulfur clusters into proteins; mitochondrial matrix protein; involved in the incorporation of iron-sulfur clusters into mitochondrial aconitase-type proteins; activates the radical-SAM family members Bio2p and Lip5p; interacts with Ccr4p in the two-hybrid system. (497 aa) |
| | MGM101 | Mitochondrial genome maintenance protein MGM101; Protein with a role in mitochondrial DNA recombinational repair; also involved in interstrand cross-link repair; binds to and catalyzes the annealing of single-stranded mtDNA; oligomerizes to form rings and filaments; related to Rad52-type recombination proteins, with limited overall similarity but sharing conserved functionally important residues; component of the mitochondrial nucleoid, required for the repair of oxidative mtDNA damage and mitochondrial genome maintenance; Belongs to the MGM101 family. (269 aa) |
| | MRP17 | Mitochondrial ribosomal protein of the small subunit; MRP17 exhibits genetic interactions with PET122, encoding a COX3-specific translational activator. (131 aa) |
| | NFU1 | NifU-like protein, mitochondrial; Protein involved in Fe-S cluster transfer to mitochondrial clients; protects [4Fe-4S] clusters from damage due to oxidative stress; acts along with Bol3 at a late step in the transfer of [4Fe-4S] clusters from the ISA complex to client proteins; Fe-S loaded homodimer at steady state; similar to NifU, a bacterial protein required for Fe/S cluster maturation; ortholog of the human NFU1, mutations of which are associated with Multiple Mitochondria Dysfunctions Syndrome (MMDS1). (256 aa) |
| | MDH1 | Mitochondrial malate dehydrogenase; catalyzes interconversion of malate and oxaloacetate; involved in the tricarboxylic acid (TCA) cycle; phosphorylated; Belongs to the LDH/MDH superfamily. MDH type 1 family. (334 aa) |
| | OCT1 | Mitochondrial intermediate peptidase; cleaves destabilizing N-terminal residues of a subset of proteins upon import, after their cleavage by mitochondrial processing peptidase (Mas1p-Mas2p); may contribute to mitochondrial iron homeostasis. (772 aa) |
| | MRPL31 | Mitochondrial ribosomal protein of the large subunit. (131 aa) |
| | RSM22 | Mitochondrial ribosomal protein of the small subunit; also predicted to be an S-adenosylmethionine-dependent RNA methyltransferase. (628 aa) |
| | MRP49 | Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation. (137 aa) |
| | MRPL38 | Mitochondrial ribosomal protein of the large subunit; appears as two protein spots (YmL34 and YmL38) on two-dimensional SDS gels; protein abundance increases in response to DNA replication stress. (138 aa) |
| | MRPL13 | Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation. (264 aa) |
| | PAM17 | Presequence translocated-associated motor subunit PAM17, mitochondrial; Constituent of the TIM23 complex; proposed alternatively to be a component of the import motor (PAM complex) or to interact with and modulate the core TIM23 (Translocase of the Inner mitochondrial Membrane) complex; protein abundance increases in response to DNA replication stress; Belongs to the PAM17 family. (197 aa) |
| | MRPL20 | Mitochondrial ribosomal protein of the large subunit. (195 aa) |
| | ISA1 | Iron-sulfur assembly protein 1; Protein required for maturation of mitochondrial [4Fe-4S] proteins; functions in a complex with Isa2p and possibly Iba57p; isa1 deletion causes loss of mitochondrial DNA and respiratory deficiency; depletion reduces growth on nonfermentable carbon sources; functional ortholog of bacterial A-type ISC proteins; human ISCA1 can complement isa1 null mutant. (250 aa) |
| | PAM18 | Subunit of the import motor (PAM complex); the PAM complex is a component of the Translocase of the Inner Mitochondrial membrane (TIM23 complex); essential J-protein cochaperone that stimulates Ssc1p ATPase activity to drive import; inhibited by Pam16p. (168 aa) |
| | SLS1 | Sigma-like sequence protein 1, mitochondrial; Mitochondrial membrane protein; coordinates expression of mitochondrially-encoded genes; may facilitate delivery of mRNA to membrane-bound translation machinery; Belongs to the SLS1 family. (643 aa) |
| | MAS1 | Beta subunit of the mitochondrial processing protease (MPP); essential processing enzyme that cleaves the N-terminal targeting sequences from mitochondrially imported proteins; Belongs to the peptidase M16 family. (462 aa) |
| | QRI5 | Mitochondrial inner membrane protein; required for accumulation of spliced COX1 mRNA; may have an additional role in translation of COX1 mRNA. (111 aa) |
| | HSP60 | Heat shock protein 60, mitochondrial; Tetradecameric mitochondrial chaperonin; required for ATP-dependent folding of precursor polypeptides and complex assembly; prevents aggregation and mediates protein refolding after heat shock; role in mtDNA transmission; phosphorylated. (572 aa) |
| | GUF1 | Translation factor GUF1, mitochondrial; Mitochondrial matrix GTPase; associates with mitochondrial ribosomes; important for translation under temperature and nutrient stress; may have a role in translational fidelity; similar to bacterial LepA elongation factor. (645 aa) |
| | ACO1 | Aconitate hydratase, mitochondrial; Aconitase; required for the tricarboxylic acid (TCA) cycle and also independently required for mitochondrial genome maintenance; component of the mitochondrial nucleoid; mutation leads to glutamate auxotrophy; human homolog ACO2 can complement yeast null mutant. (778 aa) |
| | MRPL15 | Mitochondrial ribosomal protein of the large subunit; Belongs to the ribonuclease III family. Mitochondrion- specific ribosomal protein mL57 subfamily. (253 aa) |
| | ILV5 | Ketol-acid reductoisomerase, mitochondrial; Acetohydroxyacid reductoisomerase and mtDNA binding protein; involved in branched-chain amino acid biosynthesis and maintenance of wild-type mitochondrial DNA; found in mitochondrial nucleoids. (395 aa) |
| | SSQ1 | Heat shock protein SSQ1, mitochondrial; Mitochondrial hsp70-type molecular chaperone; required for assembly of iron/sulfur clusters into proteins at a step after cluster synthesis, and for maturation of Yfh1p, which is a homolog of human frataxin implicated in Friedreich's ataxia. (657 aa) |
| | STE23 | A-factor-processing enzyme; Metalloprotease; involved in N-terminal processing of pro-a-factor to mature form; expressed in both haploids and diploids; one of two yeast homologs of human insulin-degrading enzyme (hIDE); homolog Axl1p is also involved in processing of pro-a-factor; Belongs to the peptidase M16 family. (1027 aa) |
| | MRPL4 | Mitochondrial ribosomal protein of the large subunit; homolog of prokaryotic L29 ribosomal protein; located at the ribosomal tunnel exit. (319 aa) |
| | MRPL39 | Mitochondrial ribosomal protein of the large subunit. (70 aa) |
| | YML6 | Mitochondrial ribosomal protein of the large subunit; has similarity to E. coli L4 ribosomal protein and human mitoribosomal MRP-L4 protein; essential for viability, unlike most other mitoribosomal proteins. (286 aa) |
| | AIM32 | Altered inheritance of mitochondria protein 32; Protein of unknown function; null mutant is viable and displays elevated frequency of mitochondrial genome loss. (311 aa) |
| | RPM2 | Protein subunit of mitochondrial RNase P; has roles in nuclear transcription, cytoplasmic and mitochondrial RNA processing, and mitochondrial translation; distributed to mitochondria, cytoplasmic processing bodies, and the nucleus. (1202 aa) |
| | COQ5 | 2-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrial; 2-hexaprenyl-6-methoxy-1,4-benzoquinone methyltransferase; involved in ubiquinone (Coenzyme Q) biosynthesis; localizes to the matrix face of the mitochondrial inner membrane in a large complex with other ubiquinone biosynthetic enzymes; respiratory defect of the null mutant is partially complemented by human COQ5; Belongs to the class I-like SAM-binding methyltransferase superfamily. MenG/UbiE family. (307 aa) |
| | NDI1 | Rotenone-insensitive NADH-ubiquinone oxidoreductase, mitochondrial; NADH:ubiquinone oxidoreductase; transfers electrons from NADH to ubiquinone in respiratory chain but does not pump protons, in contrast to higher eukaryotic multisubunit respiratory complex I; upon apoptotic stress, is activated in mitochondria by N-terminal cleavage, then translocates to cytoplasm to induce apoptosis; homolog of human AIFM2; yeast NDI1 complements several phenotypes of human cell line with mutated MT-ND4, implicated in Leber hereditary optic neuropathy. (513 aa) |
| | COX14 | Mitochondrial cytochrome c oxidase (complex IV) assembly factor; also involved in translational regulation of Cox1p and prevention of Cox1p aggregation before assembly; associates with complex IV assembly intermediates and complex III/complex IV supercomplexes; located in the mitochondrial membrane. (70 aa) |
| | MRPL3 | Mitochondrial ribosomal protein of the large subunit; located in close proximity to the polypeptide exit channel of the ribosome; mutations in human homolog MRPL44 cause childhood cardiomyopathy; human MRPL44 deficiency results in inefficient assembly of the mitochondrial ribosome, and in tissue-specific respiratory chain deficiency, manifesting as either Complex I+Complex IV or Complex IV deficiency, depending on a cell type; Belongs to the ribonuclease III family. Mitochondrion- specific ribosomal protein mL44 subfamily. (390 aa) |
| | ARG7 | Mitochondrial ornithine acetyltransferase; catalyzes the fifth step in arginine biosynthesis; also possesses acetylglutamate synthase activity, regenerates acetylglutamate while forming ornithine; Belongs to the ArgJ family. (441 aa) |
| | ABF2 | ARS-binding factor 2, mitochondrial; Mitochondrial DNA-binding protein; involved in mitochondrial DNA replication and recombination, member of HMG1 DNA-binding protein family; activity may be regulated by protein kinase A phosphorylation; ABF2 has a paralog, IXR1, that arose from the whole genome duplication; human homolog TFAM can complement yeast abf2 mutant, rescuing the loss-of-mitochondrial DNA phenotype in a yeast abf2 strain. (183 aa) |
| | PSO2 | DNA cross-link repair protein PSO2/SNM1; Nuclease required for DNA single- and double-strand break repair; acts at a post-incision step in repair of breaks that result from interstrand cross-links produced by a variety of mono- and bi-functional psoralen derivatives; induced by UV-irradiation; forms nuclear foci upon DNA replication stress; Belongs to the DNA repair metallo-beta-lactamase (DRMBL) family. (661 aa) |
| | ADH3 | Mitochondrial alcohol dehydrogenase isozyme III; involved in the shuttling of mitochondrial NADH to the cytosol under anaerobic conditions and ethanol production. (375 aa) |
| | MRP2 | Mitochondrial ribosomal protein of the small subunit. (115 aa) |
| | MRPL51 | Mitochondrial ribosomal protein of the large subunit. (140 aa) |
| | ISA2 | Iron-sulfur assembly protein 2; Protein required for maturation of mitochondrial [4Fe-4S] proteins; functions in a complex with Isa1p and possibly Iba57p; localizes to the mitochondrial intermembrane space, overexpression of ISA2 suppresses grx5 mutations. (185 aa) |
| | ICL2 | 2-methylisocitrate lyase of the mitochondrial matrix; functions in the methylcitrate cycle to catalyze the conversion of 2-methylisocitrate to succinate and pyruvate; ICL2 transcription is repressed by glucose and induced by ethanol. (575 aa) |
| | FUM1 | Fumarate hydratase, mitochondrial; Fumarase; converts fumaric acid to L-malic acid in the TCA cycle; cytosolic and mitochondrial distribution determined by the N-terminal targeting sequence, protein conformation, and status of glyoxylate shunt; phosphorylated in mitochondria; Belongs to the class-II fumarase/aspartase family. Fumarase subfamily. (488 aa) |
| | YAH1 | Adrenodoxin homolog, mitochondrial; Ferredoxin of the mitochondrial matrix; required for formation of cellular iron-sulfur proteins; involved in heme A biosynthesis; human homolog FDX1L can complement yeast by allowing growth during down-regulation of yeast YAH1; Belongs to the adrenodoxin/putidaredoxin family. (172 aa) |
| | CBP3 | Mitochondrial protein required for assembly of cytochrome bc1 complex; forms a complex with Cbp6p that binds to mt ribosomes near the polypeptide tunnel exit and promotes efficient translation of the COB mRNA; Cbp3p-Cbp6p complex also interacts with newly synthesized cytochrome b (Cobp) and Cbp4p to promote assembly of Cobp into the cytochrome bc1 complex; Cbp3p-Cbp6p complex is sequestered if assembly of Complex III is blocked, downregulating COB mRNA translation. (335 aa) |
| | COA2 | Cytochrome oxidase assembly factor; null mutation results in respiratory deficiency with specific loss of cytochrome oxidase activity; functions downstream of assembly factors Mss51p and Coa1p and interacts with assembly factor Shy1p; Belongs to the COA2 family. (68 aa) |
| | POS5 | Mitochondrial NADH kinase; phosphorylates NADH; also phosphorylates NAD(+) with lower specificity; required for the response to oxidative stress. (414 aa) |
| | RTC6 | 54S ribosomal protein RTC6, mitochondrial; Protein involved in translation; mutants have defects in biogenesis of nuclear ribosomes; sequence similar to prokaryotic ribosomal protein L36, may be a mitochondrial ribosomal protein; protein abundance increases in response to DNA replication stress; Belongs to the bacterial ribosomal protein bL36 family. (93 aa) |
| | MRPL40 | Mitochondrial ribosomal protein of the large subunit. (297 aa) |
| | ISU1 | Conserved protein of the mitochondrial matrix; performs a scaffolding function during assembly of iron-sulfur clusters, interacts physically and functionally with yeast frataxin (Yfh1p); ISU1 has a paralog, ISU2, that arose from the whole genome duplication; isu1 isu2 double mutant is inviable; human homolog ISCU implicated in mitochondrial myopathy, can complement isu1 isu2 double mutant; Belongs to the NifU family. (165 aa) |
| | COX11 | Cytochrome c oxidase assembly protein COX11, mitochondrial; Protein required for delivery of copper to Cox1p; mitochondrial inner membrane protein; association with mitochondrial ribosomes suggests that copper delivery may occur during translation of Cox1p; Belongs to the COX11/CtaG family. (300 aa) |
| | MRP51 | Mitochondrial ribosomal protein of the small subunit; MRP51 exhibits genetic interactions with mutations in the COX2 and COX3 mRNA 5'-untranslated leader sequences. (344 aa) |
| | GRX5 | Monothiol glutaredoxin-5, mitochondrial; Glutathione-dependent oxidoreductase; mitochondrial matrix protein involved at an early step in the biogenesis of iron-sulfur centers along with Bol1p; hydroperoxide and superoxide-radical responsive; monothiol glutaredoxin subfamily member along with Grx3p and Grx4p. (150 aa) |
| | MRPS16 | Mitochondrial ribosomal protein of the small subunit. (121 aa) |
| | ALD4 | Mitochondrial aldehyde dehydrogenase; required for growth on ethanol and conversion of acetaldehyde to acetate; phosphorylated; activity is K+ dependent; utilizes NADP+ or NAD+ equally as coenzymes; expression is glucose repressed; can substitute for cytosolic NADP-dependent aldehyde dehydrogenase when directed to the cytosol; human homolog ALDH2 can complement yeast ald4 mutant. (519 aa) |
| | MSC6 | Multicopy suppressor of HER2 involved in mitochondrial translation; mutant is defective in directing meiotic recombination events to homologous chromatids. (692 aa) |
| | MNE1 | Protein involved in splicing Group I aI5-beta intron from COX1 mRNA; mitochondrial matrix protein. (663 aa) |
| | MIP1 | Mitochondrial DNA polymerase gamma; single subunit of mitochondrial DNA polymerase in yeast, in contrast to metazoan complex of catalytic and accessory subunits; polymorphic in yeast, petites occur more frequently in some lab strains; human ortholog POLG complements yeast mip1 mutant; mutations in human POLG associated with Alpers-Huttenlocher syndrome (AHS), progressive external ophthalmoplegia (PEO), parkinsonism, other mitochondrial diseases. (1254 aa) |
| | MGE1 | GrpE protein homolog, mitochondrial; Mitochondrial matrix cochaperone; nucleotide release factor for Ssc1p in protein translocation and folding; also acts as cochaperone for Ssq1p in folding of Fe-S cluster proteins; acts as oxidative sensor to regulate mitochondrial Ssc1p; in presence of oxidative stress, dimeric Mge1p becomes a monomer and unable to regulate Ssc1p function; homolog of E. coli GrpE and human Mge1 (GRPEL1), which also responds to oxidative stress; Belongs to the GrpE family. (228 aa) |
| | ISU2 | Mitochondrial protein required for iron-sulfur protein synthesis; performs scaffolding function during Fe/S cluster assembly; involved in Fe-S cluster assembly for both mitochondrial and cytosolic proteins; protein abundance increases under DNA replication stress; ISU2 has a paralog, ISU1, that arose from the whole genome duplication; isu1 isu2 double mutant is inviable; human homolog ISCU implicated in mitochondrial myopathy, can complement isu1 isu2 double mutant. (156 aa) |
| | PET123 | Mitochondrial ribosomal protein of the small subunit; PET123 exhibits genetic interactions with PET122, which encodes a COX3 mRNA-specific translational activator. (318 aa) |
| | MRPL23 | Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2. (163 aa) |
| | LSC1 | Alpha subunit of succinyl-CoA ligase; succinyl-CoA ligase is a mitochondrial enzyme of the TCA cycle that catalyzes the nucleotide-dependent conversion of succinyl-CoA to succinate; phosphorylated. (329 aa) |
| | IDH2 | Subunit of mitochondrial NAD(+)-dependent isocitrate dehydrogenase; complex catalyzes the oxidation of isocitrate to alpha-ketoglutarate in the TCA cycle; phosphorylated; Belongs to the isocitrate and isopropylmalate dehydrogenases family. (369 aa) |
| | GLO4 | Hydroxyacylglutathione hydrolase, mitochondrial; Mitochondrial glyoxalase II; catalyzes the hydrolysis of S-D-lactoylglutathione into glutathione and D-lactate; GLO4 has a paralog, GLO2, that arose from the whole genome duplication. (285 aa) |
| | DDL1 | DDHD domain-containing phospholipase A1; mitochondrial matrix enzyme with sn-1-specific activity, hydrolyzing cardiolipin, PE, PC, PG and PA; implicated in remodeling of mitochondrial phospholipids; antagonistically regulated by Aft1p and Aft2p; in humans, mutations in DDHD1 and DDHD2 genes cause specific types of hereditary spastic paraplegia, while DDL1-defective yeast share similar phenotypes such as mitochondrial dysfunction and defects in lipid metabolism. (715 aa) |
| | HSP10 | 10 kDa heat shock protein, mitochondrial; Mitochondrial matrix co-chaperonin; inhibits the ATPase activity of Hsp60p, a mitochondrial chaperonin; involved in protein folding and sorting in the mitochondria; 10 kD heat shock protein with similarity to E. coli groES. (106 aa) |
| | ARG8 | Acetylornithine aminotransferase, mitochondrial; Acetylornithine aminotransferase; catalyzes the fourth step in the biosynthesis of the arginine precursor ornithine; Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family. (423 aa) |
| | PTH4 | Putative uncharacterized protein YOL114C; Protein similar to the human peptidyl-tRNA hydrolase gene ICT1; associates with mitochondrial large subunit; may function in translation termination; YOL114C is not an essential gene. (202 aa) |
| | HMI1 | Mitochondrial inner membrane localized ATP-dependent DNA helicase; required for the maintenance of the mitochondrial genome; not required for mitochondrial transcription; has homology to E. coli helicase uvrD. (706 aa) |
| | SDH5 | Succinate dehydrogenase assembly factor 2, mitochondrial; Protein required for flavinylation of Sdh1p; binds to Sdh1p and promotes FAD cofactor attachment, which is necessary for succinate dehydrogenase (SDH) complex assembly and activity; mutations in human ortholog PGL2 are associated with neuroendocrine tumors (paraganglioma). (162 aa) |
| | RSM19 | Mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S19 ribosomal protein. (91 aa) |
| | MRPS12 | 37S ribosomal protein S12, mitochondrial; Mitochondrial protein; may interact with ribosomes based on co-purification experiments; similar to E. coli and human mitochondrial S12 ribosomal proteins; Belongs to the universal ribosomal protein uS12 family. (153 aa) |
| | MRPL50 | Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation. (139 aa) |
| | MDJ2 | Mitochondrial DnaJ homolog 2; Constituent of the mitochondrial import motor; associated with the presequence translocase; function overlaps with that of Pam18p; stimulates the ATPase activity of Ssc1p to drive mitochondrial import; contains a J domain. (146 aa) |
| | ZIM17 | Protein co-chaperone with a zinc finger motif; essential for protein import into mitochondria; may act with Pam18p to facilitate recognition and folding of imported proteins by Ssc1p (mtHSP70) in the mitochondrial matrix; required for the maintenance of Ssc1p solubility and assists in the functional interaction of Ssc1p with substrate proteins. (174 aa) |
| | MRPS18 | Mitochondrial ribosomal protein of the small subunit; essential for viability, unlike most other mitoribosomal proteins. (217 aa) |
| | MRX6 | MIOREX complex component 6; Protein that associates with mitochondrial ribosome. (524 aa) |
| | MRPL10 | Mitochondrial ribosomal protein of the large subunit; appears as two protein spots (YmL10 and YmL18) on two-dimensional SDS gels. (322 aa) |
| | MRPL17 | Mitochondrial ribosomal protein of the large subunit. (281 aa) |
| | MRPL19 | Mitochondrial ribosomal protein of the large subunit. (158 aa) |
| | MRPL22 | Mitochondrial ribosomal protein of the large subunit. (309 aa) |
| | NAM9 | 37S ribosomal protein NAM9, mitochondrial; Mitochondrial ribosomal component of the small subunit; Belongs to the universal ribosomal protein uS4 family. (486 aa) |
| | YNL122C | Mitochondrial ribosomal protein of the large subunit; homologous to bacterial L35 and human MRPL35 ribosomal proteins. (115 aa) |
| | SWS2 | Putative mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S13 ribosomal protein; participates in controlling sporulation efficiency; localizes to vacuole in response to H2O2. (143 aa) |
| | LAT1 | Dihydrolipoamide acetyltransferase component (E2) of the PDC; the pyruvate dehydrogenase complex (PDC) catalyzes the oxidative decarboxylation of pyruvate to acetyl-CoA. (482 aa) |
| | IDH1 | Subunit of mitochondrial NAD(+)-dependent isocitrate dehydrogenase; complex catalyzes the oxidation of isocitrate to alpha-ketoglutarate in the TCA cycle; Belongs to the isocitrate and isopropylmalate dehydrogenases family. (360 aa) |
| | MRP7 | Mitochondrial ribosomal protein of the large subunit. (371 aa) |
| | MRPL33 | Mitochondrial ribosomal protein of the large subunit. (86 aa) |
| | YHM2 | Citrate and oxoglutarate carrier protein; exports citrate from and imports oxoglutarate into the mitochondrion, causing net export of NADPH reducing equivalents; also associates with mt nucleoids and has a role in replication and segregation of the mt genome; Belongs to the mitochondrial carrier (TC 2.A.29) family. (314 aa) |
| | MTF1 | Mitochondrial RNA polymerase specificity factor; has structural similarity to S-adenosylmethionine-dependent methyltransferases and functional similarity to bacterial sigma-factors; Mtf1p interacts with and stabilizes the Rpo41p-promoter complex, enhancing DNA bending and melting to facilitate pre-initiation open complex formation; Belongs to the class I-like SAM-binding methyltransferase superfamily. rRNA adenine N(6)-methyltransferase family. (341 aa) |
| | MRPL44 | Mitochondrial ribosomal protein of the large subunit; protein abundance increases in response to DNA replication stress. (98 aa) |
| | MRPL24 | Mitochondrial ribosomal protein of the large subunit; two mitochondrial ribosomal proteins, YmL14 and YmL24, have been assigned to the same gene. (258 aa) |
| | MRPS17 | Mitochondrial ribosomal protein of the small subunit. (237 aa) |
| | MRPS8 | Mitochondrial ribosomal protein of the small subunit. (155 aa) |
| | ATP6 | Subunit a of the F0 sector of mitochondrial F1F0 ATP synthase; mitochondrially encoded; translation is specifically activated by Atp22p; ATP6 and ATP8 mRNAs are not translated in the absence of the F1 sector of ATPase; mutations in human ortholog MT-ATP6 are associated with neurodegenerative disorders such as Neurogenic Ataxia and Retinitis Pigmentosa (NARP), Leigh syndrome (LS), Charcot-Marie-Tooth (CMT), and ataxia telangiectasia. (259 aa) |
| | COB | Cytochrome b; mitochondrially encoded subunit of the ubiquinol-cytochrome c reductase complex which includes Cobp, Rip1p, Cyt1p, Cor1p, Qcr2p, Qcr6p, Qcr7p, Qcr8p, Qcr9p, and Qcr10p. (385 aa) |
| | OLI1 | F0-ATP synthase subunit c (ATPase-associated proteolipid); encoded on the mitochondrial genome; mutation confers oligomycin resistance; expression is specifically dependent on the nuclear genes AEP1 and AEP2. (76 aa) |
| | VAR1 | Mitochondrial ribosomal protein of the small subunit; mitochondrially-encoded; polymorphic in different strains due to variation in number of AAT (asparagine) codons; translated near the mitochondrial inner membrane; may have a role in loss of mitochondrial DNA under stress conditions. (398 aa) |
| | BOL1 | BolA-like protein 1; Mitochondrial matrix protein involved in Fe-S cluster biogenesis; facilitates [4Fe-2S] cluster inception into mitochondrial proteins such as lipoate synthase and succinate dehydrogenase; interacts and may function with Grx5p at an early step in Fe-S cluster biosynthesis; forms dimeric complexes with Grx5p and Nfu1p that alter the stability of shared Fe/S clusters; sequence similarity to human BOLA family member, BOLA1 and S. pombe uvi31, a putative DNA repair protein. (110 aa) |
| | BOL3 | BolA-like protein 3; Protein involved in Fe-S cluster transfer to mitochondrial clients; protects [4Fe-4S] clusters from damage due to oxidative stress by acting along with Nfu1p at a late step in the transfer of [4Fe-4S] clusters from the ISA complex to mitochondrial client proteins like lipoate synthase and succinate dehydrogenase; sequence similarity to human BOLA family member, BOLA3, mutations of which are associated with Multiple Mitochondria Dysfunctions Syndrome (MMDS2). (118 aa) |
| | PIM1 | ATP-dependent Lon protease; involved in degradation of misfolded proteins in mitochondria; required for biogenesis and maintenance of mitochondria; Belongs to the peptidase S16 family. (1133 aa) |
| | MRPL16 | Mitochondrial ribosomal protein of the large subunit; homologous to bacterial L16 ribosomal protein; synthetic lethality with hac1 mutation suggests a possible role in synthesis of precursors for protein glycosylation. (232 aa) |
| | MRP21 | Mitochondrial ribosomal protein of the small subunit; MRP21 exhibits genetic interactions with mutations in the COX2 and COX3 mRNA 5'-untranslated leader sequences. (177 aa) |
| | ATP1 | Alpha subunit of the F1 sector of mitochondrial F1F0 ATP synthase; which is a large, evolutionarily conserved enzyme complex required for ATP synthesis; F1 translationally regulates ATP6 and ATP8 expression to achieve a balanced output of ATP synthase genes encoded in nucleus and mitochondria; phosphorylated; N-terminally propionylated in vivo; Belongs to the ATPase alpha/beta chains family. (545 aa) |
| | MIS1 | C-1-tetrahydrofolate synthase, mitochondrial; Mitochondrial C1-tetrahydrofolate synthase; involved in interconversion between different oxidation states of tetrahydrofolate (THF); provides activities of formyl-THF synthetase, methenyl-THF cyclohydrolase, and methylene-THF dehydrogenase. (975 aa) |
| | CBP6 | Cytochrome B pre-mRNA-processing protein 6; Mitochondrial protein required for translation of the COB mRNA; forms a complex with Cbp3p that binds to mt ribosomes near the polypeptide tunnel exit and promotes efficient translation of the COB mRNA; Cbp3p-Cbp6p complex also interacts with newly synthesized cytochrome b (Cobp) and Cbp4p to promote assembly of Cobp into the cytochrome bc1 complex; Cbp3p-Cbp6p complex is sequestered if assembly of Complex III is blocked, downregulating COB mRNA translation. (162 aa) |
| | MRPL36 | Mitochondrial ribosomal protein of the large subunit; overproduction suppresses mutations in the COX2 leader peptide-encoding region. (177 aa) |
| | MRPS9 | Mitochondrial ribosomal protein of the small subunit. (278 aa) |
| | PDB1 | E1 beta subunit of the pyruvate dehydrogenase (PDH) complex; PDH is an evolutionarily conserved multi-protein complex found in mitochondria. (366 aa) |
| | MCX1 | ATP-dependent clpX-like chaperone, mitochondrial; Non-proteolytic ATPase of the AAA family; stimulates incorporation of the pyridoxal phosphate cofactor into Hem1p (5-aminolevulinic acid synthase); localized to the mitochondrial matrix; ortholog of vertebrate CLPX, which promotes erythropoiesis. (520 aa) |
| | MRPS5 | Mitochondrial ribosomal protein of the small subunit. (307 aa) |
| | MRPL37 | Mitochondrial ribosomal protein of the large subunit. (105 aa) |
| | SDH8 | Protein required for assembly of succinate dehydrogenase; interacts with flavinylated Sdh1p and may function as a chaperone for free Sdh1p, protecting its FAD cofactor from redox reactions before assembly of the complex; soluble protein of the mitochondrial matrix; respiratory defect of null mutant is functionally complemented by Drosophila and human orthologs. (138 aa) |
| | MRPL27 | Mitochondrial ribosomal protein of the large subunit; homolog of human Bcl-2 interacting protein BMRP. (146 aa) |
| | ILV6 | Acetolactate synthase small subunit, mitochondrial; Regulatory subunit of acetolactate synthase; acetolactate synthase catalyzes the first step of branched-chain amino acid biosynthesis; enhances activity of the Ilv2p catalytic subunit, localizes to mitochondria. (309 aa) |
| | CHA1 | Catabolic L-serine/threonine dehydratase; Catabolic L-serine (L-threonine) deaminase; catalyzes the degradation of both L-serine and L-threonine; required to use serine or threonine as the sole nitrogen source, transcriptionally induced by serine and threonine; Belongs to the serine/threonine dehydratase family. (360 aa) |
| | MRPL32 | Mitochondrial ribosomal protein of the large subunit; protein abundance increases in response to DNA replication stress. (183 aa) |
| | RIM1 | ssDNA-binding protein essential for mitochondrial genome maintenance; involved in mitochondrial DNA replication; stimulates utilization by Mip1p DNA polymerase of RNA primers synthesized by Rpo41p. (135 aa) |
| | IMG1 | Mitochondrial ribosomal protein of the large subunit; required for respiration and for maintenance of the mitochondrial genome. (169 aa) |
| | IMG2 | Mitochondrial ribosomal protein of the large subunit; conserved in metazoa, with similarity to human mitochondrial ribosomal protein MRPL49. (146 aa) |
| | PTC6 | [Pyruvate dehydrogenase [acetyl-transferring]]-phosphatase 2, mitochondrial; Mitochondrial type 2C protein phosphatase (PP2C); has similarity to mammalian PP1Ks; involved in mitophagy; null mutant is sensitive to rapamycin and has decreased phosphorylation of the Pda1 subunit of pyruvate dehydrogenase. (442 aa) |
| | MRP10 | Mitochondrial ribosomal protein of the small subunit; contains twin cysteine-x9-cysteine motifs; oxidized by Mia40p during import into mitochondria. (95 aa) |
| | RAD59 | Protein involved DNA double-strand break repair; repairs breaks in DNA during vegetative growth via recombination and single-strand annealing; anneals complementary single-stranded DNA; forms nuclear foci upon DNA replication stress; required for loading of Rad52p to DSBs; regulates replication fork progression in DNA ligase I-deficient cells; paralog of Rad52p; Belongs to the RAD52 family. (238 aa) |
| | IDP1 | Mitochondrial NADP-specific isocitrate dehydrogenase; catalyzes the oxidation of isocitrate to alpha-ketoglutarate; not required for mitochondrial respiration and may function to divert alpha-ketoglutarate to biosynthetic processes. (428 aa) |
| | CBS1 | Mitochondrial translational activator of the COB mRNA; membrane protein that interacts with translating ribosomes, acts on the COB mRNA 5'-untranslated leader. (229 aa) |
| | MSS2 | Protein MSS2, mitochondrial; Peripherally bound inner membrane protein of the mitochondrial matrix; involved in membrane insertion of C-terminus of Cox2p, interacts genetically and physically with Cox18p. (351 aa) |
| | YFH1 | Frataxin homolog intermediate form; Mitochondrial matrix iron chaperone; oxidizes and stores iron; interacts with Isu1p to promote Fe-S cluster assembly; mutation results in multiple Fe/S-dependent enzyme deficiencies; human frataxin homolog FXN is mutated in Friedrich's ataxia; human FTL gene can complement yeast yfh1 null mutant. (174 aa) |
| | DLD2 | D-2-hydroxyglutarate--pyruvate transhydrogenase DLD2; D-2-hydroxyglutarate dehydrogenase, and minor D-lactate dehydrogenase; mitochondrial matrix protein that oxidizes D-2-hydroxyglutarate (D-2HG), an oncometabolite, to alpha-ketoglutarate with a minor role in lactate catabolism; located in the mitochondrial matrix; Belongs to the FAD-binding oxidoreductase/transferase type 4 family. (530 aa) |
| | MRPL11 | Mitochondrial ribosomal protein of the large subunit; localizes to vacuole in response to H2O2. (249 aa) |
| | EHD3 | 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; 3-hydroxyisobutyryl-CoA hydrolase; member of a family of enoyl-CoA hydratase/isomerases; non-tagged protein is detected in highly purified mitochondria in high-throughput studies; phosphorylated; mutation affects fluid-phase endocytosis. (500 aa) |
| | RSM10 | Mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S10 ribosomal protein; essential for viability, unlike most other mitoribosomal proteins. (203 aa) |
| | MRX14 | Putative mitochondrial ribosomal protein of the large subunit; similar to E. coli L34 ribosomal protein; required for respiratory growth, as are most mitochondrial ribosomal proteins; protein increases in abundance and relocalizes to the plasma membrane upon DNA replication stress. (105 aa) |
| | MRPL1 | Mitochondrial ribosomal protein of the large subunit. (285 aa) |
| | KGD2 | 2-oxoglutarate dehydrogenase E2 component (dihydrolipoamide succinyltransferase); Dihydrolipoyl transsuccinylase; component of the mitochondrial alpha-ketoglutarate dehydrogenase complex, which catalyzes the oxidative decarboxylation of alpha-ketoglutarate to succinyl-CoA in the TCA cycle; phosphorylated. (463 aa) |
| | RSM24 | Mitochondrial ribosomal protein of the small subunit. (319 aa) |
| | MSS116 | ATP-dependent RNA helicase MSS116, mitochondrial; Mitochondrial transcription elongation factor; DEAD-box protein; required for efficient splicing of mitochondrial Group I and II introns; non-polar RNA helicase that also facilities strand annealing; promotes RNA folding by stabilizing an early assembly intermediate. (664 aa) |
| | CBS2 | Mitochondrial translational activator of the COB mRNA; interacts with translating ribosomes, acts on the COB mRNA 5'-untranslated leader. (389 aa) |
| | HEM1 | 5-aminolevulinate synthase, mitochondrial; 5-aminolevulinate synthase; catalyzes the first step in the heme biosynthetic pathway; an N-terminal signal sequence is required for localization to the mitochondrial matrix; expression is regulated by Hap2p-Hap3p; has a pyridoxal phosphate cofactor whose insertion is mediated by Mcx1p; Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family. (548 aa) |
| | MRPL7 | Mitochondrial ribosomal protein of the large subunit; MRPL7 produces both YmL5 and YmL7, which are two different modified forms of the same protein. (292 aa) |
| | CTA1 | Catalase A; breaks down hydrogen peroxide in the peroxisomal matrix formed by acyl-CoA oxidase (Pox1p) during fatty acid beta-oxidation; Belongs to the catalase family. (515 aa) |
| | HSP78 | Heat shock protein 78, mitochondrial; Oligomeric mitochondrial matrix chaperone; cooperates with Ssc1p in mitochondrial thermotolerance after heat shock; able to prevent the aggregation of misfolded proteins as well as resolubilize protein aggregates. (811 aa) |
| | MHR1 | Mitochondrial ribosomal protein of the large subunit; also involved in homologous recombination in mitochondria; required for recombination-dependent mtDNA partitioning; involved in stimulation of mitochondrial DNA replication in response to oxidative stress. (226 aa) |
| | MRPL35 | Mitochondrial ribosomal protein of the large subunit; Belongs to the phosphatidylethanolamine-binding protein family. Mitochondrion-specific ribosomal protein mL38 subfamily. (367 aa) |
| | IRC3 | Putative ATP-dependent helicase IRC3; Double-stranded DNA-dependent helicase of the DExH/D-box family; required for maintenance of the mitochondrial (mt) genome; null mutant accumulates double-stranded breaks in mt DNA; localizes to the mt matrix; Belongs to the helicase family. IRC3 subfamily. (689 aa) |
| | MRPS28 | Mitochondrial ribosomal protein of the small subunit. (286 aa) |
| | MRP1 | Mitochondrial ribosomal protein of the small subunit; MRP1 exhibits genetic interactions with PET122, encoding a COX3-specific translational activator, and with PET123, encoding a small subunit mitochondrial ribosomal protein. (321 aa) |
| | MRP20 | Mitochondrial ribosomal protein of the large subunit. (263 aa) |
| | MRPL28 | Mitochondrial ribosomal protein of the large subunit; protein abundance increases in response to DNA replication stress. (147 aa) |
| | MZM1 | Mitochondrial zinc maintenance protein 1, mitochondrial; Protein required for assembly of the cytochrome bc(1) complex; acts as a chaperone for Rip1p and facilitates its insertion into the complex at a late stage of assembly; localized to the mitochondrial matrix; null mutant exhibits a respiratory growth defect and reduced mitochondrial zinc levels, which is characteristic of mutations affecting bc(1) complex assembly; member of the LYR protein family; human LYRM7 is a functional ortholog. (123 aa) |
| | RSM28 | Mitochondrial ribosomal protein of the small subunit; genetic interactions suggest a possible role in promoting translation initiation. (361 aa) |
| | ECM10 | Heat shock protein of the Hsp70 family; localized in mitochondrial nucleoids, plays a role in protein translocation, interacts with Mge1p in an ATP-dependent manner; overexpression induces extensive mitochondrial DNA aggregations; ECM10 has a paralog, SSC1, that arose from the whole genome duplication. (644 aa) |
| | RML2 | Mitochondrial ribosomal protein of the large subunit (L2); has similarity to E. coli L2 ribosomal protein; mutant allele (fat21) causes inability to utilize oleate, and induce oleic acid oxidation; may interfere with activity of the Adr1p transcription factor. (393 aa) |
| | ISD11 | Protein ISD11; Cysteine desulfurase (Nfs1p) activator; essential for the formation of the persulfide intermediate at the desulfurase active site during pyridoxal phosphate-dependent desulfuration of cysteine; required for mitochondrial iron-sulfur cluster biosynthesis; exclusive to eukaryotes, implicated as eukaryotic supplement to the bacterium-derived Fe-S cluster (ISC) assembly apparatus; involved in regulation of iron metabolism; member of the LYR protein family. (94 aa) |
| | RSM18 | Mitochondrial ribosomal protein of the small subunit; has similarity to E. coli S18 ribosomal protein. (138 aa) |
| | PET117 | Protein PET117, mitochondrial; Protein required for assembly of cytochrome c oxidase; Belongs to the PET117 family. (107 aa) |
| | ARG5,6 | Acetylglutamate kinase and N-acetyl-gamma-glutamyl-phosphate reductase; N-acetyl-L-glutamate kinase (NAGK) catalyzes the 2nd and N-acetyl-gamma-glutamyl-phosphate reductase (NAGSA), the 3rd step in arginine biosynthesis; synthesized as a precursor which is processed in the mitochondrion to yield mature NAGK and NAGSA; enzymes form a metabolon complex with Arg2p; NAGK C-terminal domain stabilizes the enzymes, slows catalysis and is involved in feed-back inhibition by arginine. (863 aa) |
| | RAD51 | DNA repair protein RAD51; Strand exchange protein; forms a helical filament with DNA that searches for homology; involved in the recombinational repair of double-strand breaks in DNA during vegetative growth and meiosis; homolog of Dmc1p and bacterial RecA protein. (400 aa) |
| | CCA1 | CCA tRNA nucleotidyltransferase, mitochondrial; ATP (CTP):tRNA-specific tRNA nucleotidyltransferase; different forms targeted to the nucleus, cytosol, and mitochondrion are generated via the use of multiple transcriptional and translational start sites; human homolog TRNT1 complements yeast null mutant. (546 aa) |
| | ADK2 | GTP:AMP phosphotransferase, mitochondrial; Mitochondrial adenylate kinase; catalyzes the reversible synthesis of GTP and AMP from GDP and ADP; may serve as a back-up for synthesizing GTP or ADP depending on metabolic conditions; 3' sequence of ADK2 varies with strain background. (225 aa) |
| | PDA1 | E1 alpha subunit of the pyruvate dehydrogenase (PDH) complex; catalyzes the direct oxidative decarboxylation of pyruvate to acetyl-CoA; phosphorylated; regulated by glucose; PDH complex is concentrated in spots within the mitochondrial matrix, often near the ERMES complex and near peroxisomes. (420 aa) |
| | MDJ1 | DnaJ homolog 1, mitochondrial; Co-chaperone that stimulates HSP70 protein Ssc1p ATPase activity; involved in protein folding/refolding in the mitochodrial matrix; required for proteolysis of misfolded proteins; member of the HSP40 (DnaJ) family of chaperones. (511 aa) |
| | LPD1 | Dihydrolipoyl dehydrogenase, mitochondrial; Dihydrolipoamide dehydrogenase; the lipoamide dehydrogenase component (E3) of the pyruvate dehydrogenase and 2-oxoglutarate dehydrogenase multi-enzyme complexes; PDH complex is concentrated in spots within the mitochondrial matrix, often near the ERMES complex and near peroxisomes; LPD1 has a paralog, IRC15, that arose from the whole genome duplication; Belongs to the class-I pyridine nucleotide-disulfide oxidoreductase family. (499 aa) |
| | RPO41 | Mitochondrial RNA polymerase; single subunit enzyme similar to those of T3 and T7 bacteriophages; requires a specificity subunit encoded by MTF1 for promoter recognition; Mtf1p interacts with and stabilizes the Rpo41p-promoter complex, enhancing DNA bending and melting to facilitate pre-initiation open complex formation; Rpo41p also synthesizes RNA primers for mitochondrial DNA replication. (1351 aa) |
| | YMR31 | 37S ribosomal protein YMR-31, mitochondrial; Subunit of the mitochondrial alpha-ketoglutarate dehydrogenase; recruits E3 subunit (Lpd1p) to the E1-E2 (Kgd1p, Kgd2p) core; has similarity to human mitochondrial ribosomal protein MRP-S36. (123 aa) |
| | JAC1 | J-type co-chaperone JAC1, mitochondrial; Specialized J-protein that functions in Fe-S cluster biogenesis; functions with Hsp70 in Fe-S cluster biogenesis in mitochondria; involved in iron metabolism; contains a J domain typical to J-type chaperones; localizes to the mitochondrial matrix. (184 aa) |
| | MRH4 | Mitochondrial ATP-dependent RNA helicase of the DEAD-box family; required for assembly of the large subunit of mitochondrial ribosomes; binds to the large subunit rRNA, 21S_rRNA; localizes to the matrix face of the mitochondrial inner membrane and associates with the large subunit precursor and with mature ribosomes. (561 aa) |
| | MNP1 | Mitochondrial ribosomal protein of the large subunit; has similarity to E. coli L7/L12 and human MRPL7 ribosomal proteins; associates with the mitochondrial nucleoid; required for normal respiratory growth. (194 aa) |
| | COQ8 | Atypical kinase COQ8, mitochondrial; ATPase required for ubiquinone biosynthesis and respiratory growth; maintains levels of CoQ biosynthetic proteins; binds to CoQ biosynthesis intermediates; UbiB protein kinase-like family member that lacks canonical protein kinase activity; similar to prokaryotic proteins involved in ubiquinone biosynthesis; human homolog ADCK3 complements a coq8 null, is associated with CoQ and respiratory-chain deficiencies, and is mutated in autosomal-recessive cerebellar ataxia type 2. (501 aa) |
| | RSM23 | Mitochondrial ribosomal protein of the small subunit; has similarity to mammalian apoptosis mediator proteins; null mutation prevents induction of apoptosis by overproduction of metacaspase Mca1p. (450 aa) |
| | TAM41 | Mitochondrial phosphatidate cytidylyltransferase (CDP-DAG synthase); required for cardiolipin biosynthesis; viability of null mutant is strain-dependent; mRNA is targeted to the bud; mutant displays defect in mitochondrial protein import, likely due to altered membrane lipid composition; Belongs to the TAM41 family. (385 aa) |
| | MRPL25 | Mitochondrial ribosomal protein of the large subunit; mutation confers increased replicative lifespan. (157 aa) |
| | MRP13 | Mitochondrial ribosomal protein of the small subunit. (339 aa) |
| | MRPS35 | Mitochondrial ribosomal protein of the small subunit; null mutant does not grow on glycerol, is sensitive to 2,4-dichlorophenol, and accumulates large lipid droplets. (345 aa) |
| | PDX1 | E3-binding protein of the mitochondrial pyruvate dehydrogenase complex; plays a structural role in the complex by binding and positioning dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide acetyltransferase (E2) core. (410 aa) |
| | RSM27 | Mitochondrial ribosomal protein of the small subunit. (110 aa) |
| | MRPL9 | Mitochondrial ribosomal protein of the large subunit. (269 aa) |
| | PET54 | Protein PET54; Mitochondrial inner membrane protein; binds to the 5' UTR of the COX3 mRNA to activate its translation together with Pet122p and Pet494p; also binds to the COX1 Group I intron AI5 beta to facilitate exon ligation during splicing. (293 aa) |
| | YHB1 | Flavohemoprotein; Nitric oxide oxidoreductase; flavohemoglobin that plays role in oxidative and nitrosative stress responses; protects against nitration of cellular targets and against cell growth inhibition under aerobic or anaerobic conditions; yeast flavohemoglobin Yhb1p and human homolog neuroglobin NGB protect cells against alpha-synuclein cytotoxicity and aggregate formation; protein increases in abundance, relocalizes from nucleus to cytoplasmic foci upon DNA replication stress; Belongs to the globin family. Two-domain flavohemoproteins subfamily. (399 aa) |
| | MRP4 | Mitochondrial ribosomal protein of the small subunit. (394 aa) |
| | SOD2 | Mitochondrial manganese superoxide dismutase; protects cells against oxygen toxicity and oxidative stress; human mitochondrial SOD2 can complement a yeast null mutant and human cytoplasmic SOD1 can also complement when targeted to the mitochondrial matrix. (233 aa) |
| | MAS2 | Alpha subunit of the mitochondrial processing protease (MPP); essential processing enzyme that cleaves the N-terminal targeting sequences from mitochondrially imported proteins. (482 aa) |
