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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) | ||||
ATP8 | ATP synthase protein 8; Subunit 8 of the F0 sector of mitochondrial F1F0 ATP synthase; encoded on the mitochondrial genome; ATP8 and ATP6 mRNAs are not translated in the absence of the F1 sector of ATPase. (48 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) | ||||
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) | ||||
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) | ||||
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) | ||||
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) | ||||
ATP5 | Subunit 5 of the stator stalk of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; homologous to bovine subunit OSCP (oligomycin sensitivity-conferring protein); phosphorylated; Belongs to the ATPase delta chain family. (212 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) | ||||
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) | ||||
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) | ||||
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) | ||||
ATP7 | Subunit d of the stator stalk of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis. (174 aa) | ||||
ATP14 | Subunit h of the F0 sector of mitochondrial F1F0 ATP synthase; F1F0 ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; protein abundance increases in response to DNA replication stress. (124 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) | ||||
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) | ||||
ATP4 | Subunit b of the stator stalk of mitochondrial F1F0 ATP synthase; ATP synthase is a large, evolutionarily conserved enzyme complex required for ATP synthesis; contributes to the oligomerization of the complex, which in turn determines the shape of inner membrane cristae; phosphorylated; Belongs to the eukaryotic ATPase B chain family. (244 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) | ||||
ATP15 | Epsilon 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 eukaryotic ATPase epsilon family. (62 aa) |