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| TIM50 | Essential component of the TIM23 complex; acts as receptor for the translocase of the inner mitochondrial membrane (TIM23) complex guiding incoming precursors from the TOM complex; may control the gating of the Tim23p-Tim17p channel. (476 aa) | ||||
| TIM23 | Essential component of the TIM23 complex; involved in protein import into mitochondrial matrix and inner membrane; with Tim17p, contributes to architecture and function of the import channel; TIM23 complex is short for the translocase of the inner mitochondrial membrane. (222 aa) | ||||
| CYB2 | Cytochrome b2, mitochondrial; Cytochrome b2 (L-lactate cytochrome-c oxidoreductase); component of the mitochondrial intermembrane space, required for lactate utilization; expression is repressed by glucose and anaerobic conditions. (591 aa) | ||||
| UPS1 | Protein UPS1, mitochondrial; Phosphatidic acid transfer protein; plays a role in phospholipid metabolism by transporting phosphatidic acid from the outer to the inner mitochondrial membrane; localizes to the mitochondrial intermembrane space; null mutant has altered cardiolipin and phosphatidic acid levels; ortholog of human PRELI; Belongs to the slowmo family. (175 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) | ||||
| CCP1 | Mitochondrial cytochrome-c peroxidase; degrades reactive oxygen species in mitochondria, involved in the response to oxidative stress. (361 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) | ||||
| TIM17 | Mitochondrial import inner membrane translocase subunit TIM17; Essential component of the TIM23 complex; with Tim23p, contributes to the architecture and function of the import channel; may link the import motor to the core Translocase of the Inner Mitochondrial membrane (TIM23 complex); Belongs to the Tim17/Tim22/Tim23 family. (158 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) | ||||
| MTG2 | GTPase MTG2, mitochondrial; Putative GTPase; member of the Obg family; peripheral protein of the mitochondrial inner membrane that associates with the large ribosomal subunit; required for mitochondrial translation, possibly via a role in ribosome assembly; Belongs to the TRAFAC class OBG-HflX-like GTPase superfamily. OBG GTPase family. (518 aa) | ||||
| PCP1 | Rhomboid protein 1, mitochondrial; Mitochondrial serine protease; required for the processing of various mitochondrial proteins and maintenance of mitochondrial DNA and morphology; belongs to the rhomboid-GlpG superfamily of intramembrane peptidases. (346 aa) | ||||
| TOM70 | Mitochondrial import receptor subunit TOM70; Component of the TOM (translocase of outer membrane) complex; involved in the recognition and initial import steps for all mitochondrially directed proteins; acts as a receptor for incoming precursor proteins; TOM70 has a paralog, TOM71, that arose from the whole genome duplication. (617 aa) | ||||
| TIM21 | Mitochondrial import inner membrane translocase subunit TIM21; Nonessential component of the TIM23 complex; interacts with the Translocase of the Outer Mitochondrial membrane (TOM complex) and with respiratory enzymes; may regulate the Translocase of the Inner Mitochondrial membrane (TIM23 complex) activity. (239 aa) | ||||
| COX4 | Subunit IV of cytochrome c oxidase; the terminal member of the mitochondrial inner membrane electron transport chain; precursor N-terminal 25 residues are cleaved during mitochondrial import; phosphorylated; spermidine enhances translation. (155 aa) | ||||
| UGO1 | Outer membrane component of the mitochondrial fusion machinery; binds to Fzo1p and Mgm1p to link these two GTPases during mitochondrial fusion; involved in fusion of both the outer and inner membranes; facilitates dimerization of Fzo1p during fusion; import into the outer membrane is mediated by Tom70p and Mim1p; has similarity to carrier proteins but likely not a transporter; similar to human SLC25A46 implicated in optic atroprophy spectrum disorder. (502 aa) | ||||
| UPS3 | Mitochondrial protein of unknown function; similar to Ups1p and Ups2p which are involved in regulation of mitochondrial cardiolipin and phosphatidylethanolamine levels; null is viable but interacts synthetically with ups1 and ups2 mutations; UPS3 has a paralog, UPS2, that arose from the whole genome duplication. (179 aa) | ||||
| PAA1 | Polyamine N-acetyltransferase 1; Polyamine acetyltransferase; acetylates polyamines (e.g. putrescine, spermidine, spermine) and also aralkylamines (e.g. tryptamine, phenylethylamine); may be involved in transcription and/or DNA replication; Belongs to the acetyltransferase family. AANAT subfamily. (191 aa) | ||||
| TRP1 | Phosphoribosylanthranilate isomerase; catalyzes the third step in tryptophan biosynthesis; in 2004, the sequence of TRP1 from strain S228C was updated by changing the previously annotated internal STOP (TAA) to serine (TCA); enhances vegetative growth at low and high temperatures when used as an auxotrophic marker in strains such as W303. (224 aa) | ||||
| TIM22 | Mitochondrial import inner membrane translocase subunit TIM22; Essential core component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane; forms the channel through which proteins are imported; Belongs to the Tim17/Tim22/Tim23 family. (207 aa) | ||||
| PGK1 | 3-phosphoglycerate kinase; catalyzes transfer of high-energy phosphoryl groups from the acyl phosphate of 1,3-bisphosphoglycerate to ADP to produce ATP; key enzyme in glycolysis and gluconeogenesis. (416 aa) | ||||
| LEU2 | Beta-isopropylmalate dehydrogenase (IMDH); catalyzes the third step in the leucine biosynthesis pathway; can additionally catalyze the conversion of beta-ethylmalate into alpha-ketovalerate; Belongs to the isocitrate and isopropylmalate dehydrogenases family. (364 aa) | ||||
| FZO1 | Mitofusin; integral membrane protein involved in mitochondrial outer membrane tethering and fusion; role in mitochondrial genome maintenance; efficient tethering and degradation of Fzo1p requires an intact N-terminal GTPase domain; targeted for destruction by the ubiquitin ligase SCF-Mdm30p and the cytosolic ubiquitin-proteasome system; Belongs to the TRAFAC class dynamin-like GTPase superfamily. Dynamin/Fzo/YdjA family. Mitofusin subfamily. (855 aa) | ||||
| GAL1 | Galactokinase; phosphorylates alpha-D-galactose to alpha-D-galactose-1-phosphate in the first step of galactose catabolism; expression regulated by Gal4p; human homolog GALK2 complements yeast null mutant; GAL1 has a paralog, GAL3, that arose from the whole genome duplication. (528 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) | ||||
| ADH1 | Alcohol dehydrogenase; fermentative isozyme active as homo- or heterotetramers; required for the reduction of acetaldehyde to ethanol, the last step in the glycolytic pathway; ADH1 has a paralog, ADH5, that arose from the whole genome duplication. (348 aa) | ||||
| HIS3 | Imidazoleglycerol-phosphate dehydratase; catalyzes the sixth step in histidine biosynthesis; mutations cause histidine auxotrophy and sensitivity to Cu, Co, and Ni salts; transcription is regulated by general amino acid control via Gcn4p. (220 aa) | ||||
| MGM1 | Mitochondrial GTPase, present in complex with Ugo1p and Fzo1p; required for mitochondrial morphology, fusion, and genome maintenance; promotes membrane bending; exists as long and short form with different distributions; ratio of long to short forms is regulated by Psd1p; homolog of human OPA1 involved in autosomal dominant optic atrophy. (881 aa) | ||||
| TIM18 | Mitochondrial import inner membrane translocase subunit TIM18; Component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane; may mediate assembly or stability of the complex; Belongs to the CybS family. (192 aa) | ||||