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| 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) | ||||
| COX1 | Subunit I of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain; one of three mitochondrially-encoded subunits; Belongs to the heme-copper respiratory oxidase family. (534 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) | ||||
| 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) | ||||
| COX2 | Subunit II of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain; one of three mitochondrially-encoded subunits. (251 aa) | ||||
| ATP3 | Gamma subunit of the F1 sector of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis. (311 aa) | ||||
| MBA1 | Membrane-associated mitochondrial ribosome receptor; forms a complex with Mdm38p that may facilitate recruitment of mRNA-specific translational activators to ribosomes; possible role in protein export from the matrix to inner membrane. (278 aa) | ||||
| ATP16 | Delta subunit of the central stalk of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase 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. (160 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) | ||||
| TPI1 | Triose phosphate isomerase, abundant glycolytic enzyme; mRNA half-life is regulated by iron availability; transcription is controlled by activators Reb1p, Gcr1p, and Rap1p through binding sites in the 5' non-coding region; inhibition of Tpi1p activity by PEP (phosphoenolpyruvate) stimulates redox metabolism in respiring cells; E104D mutation in human homolog TPI1 causes a rare autosomal disease; human TPI1 can complement yeast null mutant. (248 aa) | ||||
| TIM11 | Subunit e of mitochondrial F1F0-ATPase; ATPase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; essential for the dimeric and oligomeric state of ATP synthase, which in turn determines the shape of inner membrane cristae. (96 aa) | ||||
| OXA1 | Mitochondrial inner membrane insertase; mediates the insertion of both mitochondrial- and nuclear-encoded proteins from the matrix into the inner membrane; also has a role in insertion of carrier proteins into the inner membrane; acts as a voltage-gated ion channel, activated by substrate peptides; interacts with mitochondrial ribosomes; conserved from bacteria to animals. (402 aa) | ||||
| COX18 | Cytochrome c oxidase assembly protein COX18, mitochondrial; Protein required for membrane insertion of C-terminus of Cox2p; mitochondrial integral inner membrane protein; interacts genetically and physically with Mss2p and Pnt1p; similar to S. cerevisiae Oxa1, N. crassa Oxa2p, and E. coli YidC; respiratory defect of the null mutant is functionally complemented by human COX18 carrying the N-terminal 54 amino acids of S. cerevisiae Cox18p; Belongs to the OXA1/ALB3/YidC family. (316 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) | ||||
| 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) | ||||
| QCR8 | Subunit 8 of ubiquinol cytochrome-c reductase (Complex III); Complex III is a component of the mitochondrial inner membrane electron transport chain; oriented facing the intermembrane space; expression is regulated by Abf1p and Cpf1p; Belongs to the UQCRQ/QCR8 family. (94 aa) | ||||
| MRP49 | Mitochondrial ribosomal protein of the large subunit; not essential for mitochondrial translation. (137 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) | ||||
| 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) | ||||
| TOM40 | Mitochondrial import receptor subunit TOM40; Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import steps for all mitochondrially directed proteins; constitutes the core element of the protein conducting pore; pre-Tom40p is phosphorylated by PKA, which impairs its import into mitochondria under non-respiratory conditions. (387 aa) | ||||
| MRPL22 | Mitochondrial ribosomal protein of the large subunit. (309 aa) | ||||
| MDM38 | Mitochondrial distribution and morphology protein 38; Mitochondrial protein; forms a complex with Mba1p to facilitate recruitment of mRNA-specific translational activators to ribosomes; roles in protein export and K+/H+ exchange; human ortholog Letm1 implicated in Wolf-Hirschhorn syndrome. (573 aa) | ||||
| CYT1 | Cytochrome c1, heme protein, mitochondrial; Cytochrome c1; component of the mitochondrial respiratory chain; expression is regulated by the heme-activated, glucose-repressed Hap2p/3p/4p/5p CCAAT-binding complex. (309 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) | ||||
| PNT1 | Pentamidine resistance factor, mitochondrial; Mitochondrial integral inner membrane protein; involved in membrane insertion of C-terminus of Cox2p, interacts genetically and physically with Cox18p; deletion mutant sensitive to the anti-Pneumocystis carinii drug pentamidine. (423 aa) | ||||
| 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) | ||||
| YME1 | Catalytic subunit of i-AAA protease complex; complex is located in mitochondrial inner membrane; responsible for degradation of unfolded or misfolded mitochondrial gene products; serves as nonconventional translocation motor to pull PNPase into intermembrane space; also has role in intermembrane space protein folding; mutation causes elevated rate of mitochondrial turnover; human homolog YME1L1 can complement yeast null mutant; In the C-terminal section; belongs to the peptidase M41 family. (747 aa) | ||||
| YLH47 | LETM1 domain-containing protein YLH47, mitochondrial; Mitochondrial inner membrane protein; exposed to the mitochondrial matrix; associates with mitochondrial ribosomes; NOT required for respiratory growth; homolog of human Letm1, a protein implicated in Wolf-Hirschhorn syndrome. (454 aa) | ||||