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| 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) | ||||
| MTF2 | Mitochondrial transcription factor 2; Mitochondrial protein that interacts with mitochondrial RNA polymerase; interacts with an N-terminal region of mitochondrial RNA polymerase (Rpo41p) and couples RNA processing and translation to transcription. (440 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) | ||||
| 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) | ||||
| INA22 | Inner membrane assembly complex subunit 22; F1F0 ATP synthase peripheral stalk assembly factor; subunit of the matrix-exposed inner mitochondrial membrane localized INA complex (Ina22p-Ina17p) involved in assembly of the F1F0 peripheral stalk; co-purifies with Aim43p, ATP synthase subunits, and cytochrome bc1 complex assembly factors; interacts with Arh1p, a mitochondrial oxidoreductase; deletion mutant has a respiratory growth defect. (216 aa) | ||||
| NCA3 | Protein involved in mitochondrion organization; functions with Nca2p to regulate mitochondrial expression of subunits 6 (Atp6p) and 8 (Atp8p) of the Fo-F1 ATP synthase; SWAT-GFP, seamless-GFP and mCherry fusion proteins localize to the vacuole; member of the SUN family; expression induced in cells treated with the mycotoxin patulin; NCA3 has a paralog, UTH1, that arose from the whole genome duplication. (337 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) | ||||
| ATP12 | Protein ATP12, mitochondrial; Assembly factor for F1 sector of mitochondrial F1F0 ATP synthase; conserved protein; required for assembly of alpha and beta subunits into F1 sector of mitochondrial F1F0 ATP synthase; human homolog ATPAF2 can complement yeast atp12 mutant; mutation of human homolog reduces active ATP synthase levels and is associated with the disorder ATPAF2 deficiency. (325 aa) | ||||
| UTR1 | ATP-NADH kinase; phosphorylates both NAD and NADH; active as a hexamer; enhances the activity of ferric reductase (Fre1p); UTR1 has a paralog, YEF1, that arose from the whole genome duplication. (530 aa) | ||||
| ADE13 | Adenylosuccinate lyase; catalyzes two steps in the 'de novo' purine nucleotide biosynthetic pathway; expression is repressed by adenine and activated by Bas1p and Pho2p; mutations in human ortholog ADSL cause adenylosuccinase deficiency; human ADSL can complement yeast ADE13 null mutant. (482 aa) | ||||
| ATP10 | Mitochondrial ATPase complex subunit ATP10; Assembly factor for the F0 sector of mitochondrial F1F0 ATP synthase; mitochondrial inner membrane protein; interacts genetically with ATP6; Belongs to the ATP10 family. (279 aa) | ||||
| SOV1 | Mitochondrial protein of unknown function. (898 aa) | ||||
| ATP25 | ATPase synthesis protein 25, mitochondrial; Mitochondrial protein required for the stability of Oli1p (Atp9p) mRNA; also required for the Oli1p ring formation; YMR098C is not an essential gene. (612 aa) | ||||
| PET111 | Protein PET111, mitochondrial; Mitochondrial translational activator specific for the COX2 mRNA; located in the mitochondrial inner membrane. (800 aa) | ||||
| KAR1 | Cell division control protein KAR1; Protein involved in karyogamy and spindle pole body duplication; involved in karyogamy during mating; involved in spindle pole body duplication during mitosis; localizes to the half-bridge of the spindle pole body; interacts with Spc72p during karyogamy; also interacts with Cdc31p; essential gene. (433 aa) | ||||
| ATP11 | Protein ATP11, mitochondrial; Molecular chaperone; required for the assembly of alpha and beta subunits into the F1 sector of mitochondrial F1F0 ATP synthase; N-terminally propionylated in vivo. (318 aa) | ||||
| ATP23 | Putative metalloprotease of the mitochondrial inner membrane; required for processing of Atp6p; has an additional role in assembly of the F0 sector of the F1F0 ATP synthase complex; substrate of the Mia40p-Erv1p disulfide relay system, and folding is assisted by Mia40p; Belongs to the peptidase M76 family. (270 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) | ||||
| AEP3 | ATPase expression protein 3; Peripheral mitochondrial inner membrane protein; may facilitate use of unformylated tRNA-Met in mitochondrial translation initiation; stabilizes the bicistronic AAP1-ATP6 mRNA. (606 aa) | ||||
| INA17 | Inner membrane assembly complex subunit 17; F1F0 ATPase synthase peripheral stalk assembly factor; subunit of the matrix-exposed inner mitochondrial membrane localized INA complex (Ina22p-Ina17p) involved in assembly of the F1F0 peripheral stalk; co-purifies with Ina22p and ATP synthase subunits; null mutant displays elevated frequency of mitochondrial genome loss and has a respiratory growth defect. (182 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) | ||||
| NCA2 | Nuclear control of ATPase protein 2; Protein that regulates expression of Fo-F1 ATP synthase subunits; involved in the regulation of mitochondrial expression of subunits 6 (Atp6p) and 8 (Atp8p) of the Fo-F1 ATP synthase; functions with Nca3p. (616 aa) | ||||