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COX15 COX15 ATP6 ATP6 COB COB OLI1 OLI1 CYC3 CYC3 COQ1 COQ1 SCO2 SCO2 ATP16 ATP16 PET100 PET100 SDH4 SDH4 COQ4 COQ4 ARH1 ARH1 SDH6 SDH6 MZM1 MZM1 SDH7 SDH7 RIP1 RIP1 CYC7 CYC7 PET117 PET117 COQ8 COQ8 ARO8 ARO8 SHY1 SHY1 COQ6 COQ6 COX6 COX6 COX23 COX23 ARO9 ARO9 QCR8 QCR8 SDH1 SDH1 COX17 COX17 SDH2 SDH2 COQ9 COQ9 COQ11 COQ11 COQ5 COQ5 NDI1 NDI1 HFD1 HFD1 COA6 COA6 COQ2 COQ2 SDH5 SDH5 COQ3 COQ3 CAT5 CAT5 COX11 COX11 COX10 COX10 CBP3 CBP3 YAH1 YAH1 ATP15 ATP15
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COX15Cytochrome c oxidase assembly protein COX15; Protein required for the hydroxylation of heme O to form heme A; heme A is an essential prosthetic group for cytochrome c oxidase; Belongs to the COX15/CtaA family. (486 aa)
ATP6Subunit 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)
COBCytochrome 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)
OLI1F0-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)
CYC3Cytochrome c heme lyase (holocytochrome c synthase); attaches heme to apo-cytochrome c (Cyc1p or Cyc7p) in mitochondrial intermembrane space; human homolog HCCS implicated in microphthalmia with linear skin defects (MLS), and can complement yeast null mutant. (269 aa)
COQ1Hexaprenyl pyrophosphate synthetase; catalyzes the first step in ubiquinone (coenzyme Q) biosynthesis; Belongs to the FPP/GGPP synthase family. (473 aa)
SCO2Protein anchored to mitochondrial inner membrane; may have a redundant function with Sco1p in delivery of copper to cytochrome c oxidase; interacts with Cox2p; SCO2 has a paralog, SCO1, that arose from the whole genome duplication. (301 aa)
ATP16Delta 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)
PET100Protein PET100, mitochondrial; Chaperone that facilitates the assembly of cytochrome c oxidase; integral to the mitochondrial inner membrane; interacts with a subcomplex of subunits VII, VIIa, and VIII (Cox7p, Cox9p, and Cox8p) but not with the holoenzyme. (111 aa)
SDH4Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial; Membrane anchor subunit of succinate dehydrogenase (SDH); involved in coupling the oxidation of succinate to the transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; has similarity to human SDH subunit D (SDHD), which is implicated in paraganglioma. (181 aa)
COQ4Protein with a role in ubiquinone (Coenzyme Q) biosynthesis; possibly functioning in stabilization of Coq7p; located on matrix face of mitochondrial inner membrane; component of a mitochondrial ubiquinone-synthesizing complex; human homolog COQ4 can complement yeast coq4 null mutant. (335 aa)
ARH1Probable NADPH:adrenodoxin oxidoreductase, mitochondrial; Oxidoreductase of the mitochondrial inner membrane; involved in cytoplasmic and mitochondrial iron homeostasis and required for activity of Fe-S cluster-containing enzymes; one of the few mitochondrial proteins essential for viability; Belongs to the ferredoxin--NADP reductase type 1 family. (493 aa)
SDH6Mitochondrial protein involved in assembly of succinate dehydrogenase; has a role in maturation of the Sdh2p subunit; member of the LYR protein family; mutations in human ortholog SDHAF1 are associated with infantile leukoencephalopathy. (79 aa)
MZM1Mitochondrial zinc maintenance protein 1, mitochondrial; Protein required for assembly of the cytochrome bc(1) complex; acts as a chaperone for Rip1p and facilitates its insertion into the complex at a late stage of assembly; localized to the mitochondrial matrix; null mutant exhibits a respiratory growth defect and reduced mitochondrial zinc levels, which is characteristic of mutations affecting bc(1) complex assembly; member of the LYR protein family; human LYRM7 is a functional ortholog. (123 aa)
SDH7Mitochondrial protein involved in assembly of succinate dehydrogenase; has a role in maturation of the Sdh2p subunit; localized to the mitochondrial intermembrane space; required for acetate utilization and gluconeogenesis; mutation in Drosophila ortholog SDHAF3 causes reduced succinate dehydrogenase activity and neuronal and muscular dysfunction; member of the LYR protein family. (133 aa)
RIP1Ubiquinol-cytochrome-c reductase; a Rieske iron-sulfur protein of the mitochondrial cytochrome bc1 complex; transfers electrons from ubiquinol to cytochrome c1 during respiration; during import, Rip1p is first imported into the mitochondrial matrix where it is processed, acquires its Fe-S cluster, and is folded, then is translocated into the inner membrane by the action of a homo-oligomer of Bcs1p, and finally is delivered by Bcs1p to Complex III for assembly. (215 aa)
CYC7Cytochrome c isoform 2, expressed under hypoxic conditions; also known as iso-2-cytochrome c; electron carrier of the mitochondrial intermembrane space that transfers electrons from ubiquinone-cytochrome c oxidoreductase to cytochrome c oxidase during cellular respiration; protein abundance increases in response to DNA replication stress; CYC7 has a paralog, CYC1, that arose from the whole genome duplication. (113 aa)
PET117Protein PET117, mitochondrial; Protein required for assembly of cytochrome c oxidase; Belongs to the PET117 family. (107 aa)
COQ8Atypical kinase COQ8, mitochondrial; ATPase required for ubiquinone biosynthesis and respiratory growth; maintains levels of CoQ biosynthetic proteins; binds to CoQ biosynthesis intermediates; UbiB protein kinase-like family member that lacks canonical protein kinase activity; similar to prokaryotic proteins involved in ubiquinone biosynthesis; human homolog ADCK3 complements a coq8 null, is associated with CoQ and respiratory-chain deficiencies, and is mutated in autosomal-recessive cerebellar ataxia type 2. (501 aa)
ARO8Aromatic/aminoadipate aminotransferase 1; Aromatic aminotransferase I; expression is regulated by general control of amino acid biosynthesis. (500 aa)
SHY1Cytochrome 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)
COQ6Flavin-dependent monooxygenase involved in ubiquinone biosynthesis; responsible for hydroxylation at position C5 and deamination at C4 during ubiquinone (Coenzyme Q) biosynthesis; localizes to matrix face of mitochondrial inner membrane in a large complex with other ubiquinone biosynthetic enzymes; human homolog COQ6 can complement yeast null mutant and is implicated in steroid-resistant nephrotic syndrome (SRNS). (479 aa)
COX6Subunit 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)
COX23Cytochrome c oxidase-assembly factor COX23, mitochondrial; Protein that functions in mitochondrial copper homeostasis; mitochondrial intermembrane space protein; essential for functional cytochrome oxidase expression; homologous to Cox17p; contains twin cysteine-x9-cysteine motifs. (151 aa)
ARO9Aromatic aminotransferase II; catalyzes the first step of tryptophan, phenylalanine, and tyrosine catabolism; Belongs to the class-I pyridoxal-phosphate-dependent aminotransferase family. (513 aa)
QCR8Subunit 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)
SDH1Flavoprotein subunit of succinate dehydrogenase; couples the oxidation of succinate to the transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; FAD binding to Sdh1p is required for the assembly of the succinate dehydrogenase subunits; mutations in human ortholog SDHA are associated with Leigh syndrome. (640 aa)
COX17Copper metallochaperone that transfers copper to Sco1p and Cox11p; eventual delivery to cytochrome c oxidase; contains twin cysteine-x9-cysteine motifs; interacts with the MICOS complex, and interaction is promoted by copper ions; human homolog COX17 partially complements yeast null mutant. (69 aa)
SDH2Iron-sulfur protein subunit of succinate dehydrogenase; the complex couples the oxidation of succinate to the transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; other members are Sdh1p, Sdh3p, and Sdh4p. (266 aa)
COQ9Protein required for ubiquinone biosynthesis and respiratory growth; localizes to matrix face of mitochondrial inner membrane in a large complex with ubiquinone biosynthetic enzymes; ubiquinone is also known as coenzyme Q; human homolog COQ9 can complement yeast coq9 null mutant. (260 aa)
COQ11MIOREX complex component 2; Putative oxidoreductase, subunit of Coenzyme Q biosynthetic complexes; required for synthesis of wild-type levels of Coenzyme Q (ubiquinone); member of the short-chain dehydrogenase/reductase (SDR) superfamily; orthologous gene in some other fungi is fused to the COQ10 ortholog. (277 aa)
COQ52-methoxy-6-polyprenyl-1,4-benzoquinol methylase, mitochondrial; 2-hexaprenyl-6-methoxy-1,4-benzoquinone methyltransferase; involved in ubiquinone (Coenzyme Q) biosynthesis; localizes to the matrix face of the mitochondrial inner membrane in a large complex with other ubiquinone biosynthetic enzymes; respiratory defect of the null mutant is partially complemented by human COQ5; Belongs to the class I-like SAM-binding methyltransferase superfamily. MenG/UbiE family. (307 aa)
NDI1Rotenone-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)
HFD1Fatty aldehyde dehydrogenase HFD1; Dehydrogenase involved in ubiquinone and sphingolipid metabolism; oxidizes 4-hydroxybenzaldehyde into 4-hydroxybenzoic acid in ubiquinone biosynthesis; converts hexadecenal to hexadecenoic acid in sphingosine 1-phosphate breakdown pathway; located in the mitochondrial outer membrane and also in lipid particles; human homolog ALDH3A2, a fatty aldehyde dehydrogenase (FALDH) mutated in neurocutaneous disorder Sjogren-Larsson syndrome, can complement yeast hfd1 mutant. (532 aa)
COA6Protein involved in cytochrome c oxidase (Complex IV) assembly; involved in delivery of copper to Complex IV; also required for efficient formation of respiratory supercomplexes comprised of Complexes III and IV; localizes to the mitochondrial intermembrane space; ortholog implicated in cardiac defects in zebrafish and human; transcription is induced in response to the DNA-damaging agent MMS; protein abundance increases in response to DNA replication stress. (104 aa)
COQ24-hydroxybenzoate polyprenyltransferase, mitochondrial; Para hydroxybenzoate polyprenyl transferase; catalyzes the second step in ubiquinone (coenzyme Q) biosynthesis; human COQ2, mutations in which are implicated in an increased risk of mutiple-system atrophy, can complement a yeast coq2 null mutant. (372 aa)
SDH5Succinate dehydrogenase assembly factor 2, mitochondrial; Protein required for flavinylation of Sdh1p; binds to Sdh1p and promotes FAD cofactor attachment, which is necessary for succinate dehydrogenase (SDH) complex assembly and activity; mutations in human ortholog PGL2 are associated with neuroendocrine tumors (paraganglioma). (162 aa)
COQ3O-methyltransferase; catalyzes two different O-methylation steps in ubiquinone (Coenzyme Q) biosynthesis; component of a mitochondrial ubiquinone-synthesizing complex; phosphoprotein; Belongs to the class I-like SAM-binding methyltransferase superfamily. UbiG/COQ3 family. (312 aa)
CAT55-demethoxyubiquinone hydroxylase, mitochondrial; Protein required for ubiquinone (Coenzyme Q) biosynthesis; localizes to the matrix face of the mitochondrial inner membrane in a large complex with ubiquinone biosynthetic enzymes; required for gluconeogenic gene activation; Belongs to the COQ7 family. (233 aa)
COX11Cytochrome c oxidase assembly protein COX11, mitochondrial; Protein required for delivery of copper to Cox1p; mitochondrial inner membrane protein; association with mitochondrial ribosomes suggests that copper delivery may occur during translation of Cox1p; Belongs to the COX11/CtaG family. (300 aa)
COX10Protoheme IX farnesyltransferase, mitochondrial; Heme A:farnesyltransferase; catalyzes first step in conversion of protoheme to heme A prosthetic group required for cytochrome c oxidase activity; human ortholog COX10 can complement yeast cox10 null mutant; human ortholog COX10 is associated with mitochondrial disorders; Belongs to the UbiA prenyltransferase family. (462 aa)
CBP3Mitochondrial 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)
YAH1Adrenodoxin homolog, mitochondrial; Ferredoxin of the mitochondrial matrix; required for formation of cellular iron-sulfur proteins; involved in heme A biosynthesis; human homolog FDX1L can complement yeast by allowing growth during down-regulation of yeast YAH1; Belongs to the adrenodoxin/putidaredoxin family. (172 aa)
ATP15Epsilon 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)
Your Current Organism:
Saccharomyces cerevisiae
NCBI taxonomy Id: 4932
Other names: ATCC 18824, Candida robusta, Mycoderma cerevisiae, NRRL Y-12632, S. cerevisiae, Saccharomyces capensis, Saccharomyces italicus, Saccharomyces oviformis, Saccharomyces uvarum var. melibiosus, yeast
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