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| CDC28 | Cyclin-dependent kinase (CDK) catalytic subunit; master regulator of mitotic and meiotic cell cycles; alternately associates with G1, S, G2/M phase cyclins, which provide substrate specificity; regulates metabolism, basal transcription, chromosome dynamics, growth and morphogenesis; transcript induction in osmostress involves antisense RNA; human homologs CDK1, CDK2, CDK3 can complement yeast conditional cdc28 mutants; human CDK1, CDK2 can complement yeast cdc28 null mutant. (298 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) | ||||
| PDR3 | Transcription factor PDR3; Transcriptional activator of the pleiotropic drug resistance network; regulates expression of ATP-binding cassette (ABC) transporters through binding to cis-acting PDRE sites (PDR responsive elements); has a role in response to drugs and organic solvents; post-translationally up-regulated in cells lacking functional mitochondrial genome; involved in diauxic shift; relative distribution to nucleus increases upon DNA replication stress; APCC(Cdh1) substrate. (976 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) | ||||
| 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) | ||||
| ATG31 | Autophagy-related protein 31; Autophagy-specific protein required for autophagosome formation; forms a complex with Atg17p and Atg29p that localizes other proteins to the pre-autophagosomal structure; constitutively phosphorylated, and phosphorylation of residue S174 is required for function; high-copy suppressor of CIK1 deletion. (196 aa) | ||||
| CBS2 | Mitochondrial translational activator of the COB mRNA; interacts with translating ribosomes, acts on the COB mRNA 5'-untranslated leader. (389 aa) | ||||
| ATP22 | Specific translational activator for the mitochondrial ATP6 mRNA; Atp6p encodes a subunit of F1F0 ATP synthase; localized to the mitochondrial inner membrane; Belongs to the ATP22 family. (684 aa) | ||||
| QCR7 | Subunit 7 of ubiquinol cytochrome-c reductase (Complex III); Complex III is a component of the mitochondrial inner membrane electron transport chain; oriented facing the mitochondrial matrix; N-terminus appears to play a role in complex assembly; Belongs to the UQCRB/QCR7 family. (127 aa) | ||||
| PET122 | Protein PET122, mitochondrial; Mitochondrial translational activator specific for the COX3 mRNA; acts together with Pet54p and Pet494p; located in the mitochondrial inner membrane. (254 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) | ||||
| MSP1 | Protein MSP1; Highly-conserved N-terminally anchored AAA-ATPase; distributed in the mitochondrial outer membrane and peroxisomes; involved in mitochondrial protein sorting; functions as an extraction engine in local organelle surveillance to remove and initiate degradation of mistargeted proteins, ensuring fidelity of organelle-specific localization of tail-anchored proteins; contains an N-terminal transmembrane domain and C-terminal cytoplasmic ATPase domain. (362 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) | ||||
| SHY1 | Cytochrome oxidase assembly protein SHY1; Mitochondrial inner membrane protein required for complex IV assembly; associates with complex IV assembly intermediates and complex III/complex IV supercomplexes; similar to human SURF1 involved in Leigh Syndrome; complex IV is also known as cytochrome c oxidase. (389 aa) | ||||
| CBP4 | Assembly factor CBP4; Mitochondrial protein required for assembly of cytochrome bc1 complex; interacts with the Cbp3p-Cbp6p complex and newly synthesized cytochrome b (Cobp) to promote assembly of Cobp into the cytochrome bc1 complex. (170 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) | ||||
| COX6 | Subunit VI of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain; expression is regulated by oxygen levels. (148 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) | ||||
| MRX5 | MIOREX complex component 5; Protein that associates with mitochondrial ribosome; homozygous diploid deletion strain has a sporulation defect characterized by elevated dityrosine in the soluble fraction; expression induced by calcium shortage; YJL147W is a non-essential gene. (382 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) | ||||
| 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) | ||||
| ATP2 | Beta 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; Belongs to the ATPase alpha/beta chains family. (511 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) | ||||
| PET309 | Pentatricopeptide repeat-containing protein PET309, mitochondrial; Specific translational activator for the COX1 mRNA; binds to the COX1 mRNA; also influences stability of intron-containing COX1 primary transcripts; localizes to the mitochondrial inner membrane; contains 12 pentatricopeptide repeats (PPRs). (965 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) | ||||
| GAD1 | Glutamate decarboxylase; converts glutamate into gamma-aminobutyric acid (GABA) during glutamate catabolism; involved in response to oxidative stress. (585 aa) | ||||
| PET111 | Protein PET111, mitochondrial; Mitochondrial translational activator specific for the COX2 mRNA; located in the mitochondrial inner membrane. (800 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) | ||||
| GIS2 | Zinc finger protein GIS2; Translational activator for mRNAs with internal ribosome entry sites; associates with polysomes and binds to a specific subset of mRNAs; localizes to RNA processing bodies (P bodies) and to stress granules; may have a role in translation regulation under stress conditions; ortholog of human ZNF9/CNBP, a gene involved in myotonic dystrophy type 2. (153 aa) | ||||
| PET494 | Mitochondrial translational activator specific for the COX3 mRNA; acts together with Pet54p and Pet122p; located in the mitochondrial inner membrane. (489 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) | ||||
| PEP4 | Saccharopepsin; Vacuolar aspartyl protease (proteinase A); required for posttranslational precursor maturation of vacuolar proteinases; important for protein turnover after oxidative damage; plays a protective role in acetic acid induced apoptosis; synthesized as a zymogen, self-activates. (405 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) | ||||