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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) |
| | AI1 | Putative COX1/OXI3 intron 1 protein; Reverse transcriptase required for splicing of the COX1 pre-mRNA; encoded by a mobile group II intron within the mitochondrial COX1 gene. (834 aa) |
| | AI2 | Putative COX1/OXI3 intron 2 protein; Reverse transcriptase required for splicing of the COX1 pre-mRNA; encoded by a mobile group II intron within the mitochondrial COX1 gene. (854 aa) |
| | AI3 | Truncated non-functional cytochrome oxidase 1; Endonuclease I-SceIII; encoded by a mobile group I intron within the mitochondrial COX1 gene. (415 aa) |
| | AI4 | Truncated non-functional cytochrome oxidase 1; Endonuclease I-SceII; encoded by a mobile group I intron within the mitochondrial COX1 gene; intron is normally spliced by the BI4p maturase but AI4p can mutate to acquire the same maturase activity. (556 aa) |
| | AI5_ALPHA | Truncated non-functional cytochrome oxidase 1; Endonuclease I-SceIV; involved in intron mobility; encoded by a mobile group I intron within the mitochondrial COX1 gene; In the N-terminal section; belongs to the heme-copper respiratory oxidase family. (630 aa) |
| | AI5_BETA | Intron-encoded DNA endonuclease aI5 beta; Protein of unknown function; encoded within an intron of the mitochondrial COX1 gene; translational initiation codon is predicted to be ATA rather than ATG. (354 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) |
| | BI2 | Cytochrome b mRNA maturase bI2; Mitochondrial mRNA maturase with a role in splicing; encoded by both exon and intron sequences of partially processed COB mRNA; In the N-terminal section; belongs to the cytochrome b family. (423 aa) |
| | BI3 | Cytochrome b mRNA maturase bI3; Mitochondrial mRNA maturase; forms a complex with Mrs1p to mediate splicing of the bI3 intron of the COB gene; encoded by both exon and intron sequences of partially processed COB mRNA. (517 aa) |
| | BI4 | Truncated, nonfunctional cytochrome b; Mitochondrial mRNA maturase; forms a complex with Nam2p to mediate splicing of the bI4 intron of the COB gene; encoded by both exon and intron sequences of partially processed COB mRNA. (638 aa) |
| | SCEI | I-SceI DNA endonuclease; encoded by the mitochondrial group I intron of the 21S_rRNA gene; mediates gene conversion that propagates the intron into intron-less copies of the 21S_rRNA gene. (235 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) |
| | Q0255 | Uncharacterized mitochondrial protein RF1; Maturase-like protein. (472 aa) |
| | COX3 | Subunit III 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. (269 aa) |
| | CYC3 | Cytochrome 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) |
| | COR1 | Core subunit of the ubiquinol-cytochrome c reductase complex; the ubiquinol-cytochrome c reductase complex (bc1 complex) is a component of the mitochondrial inner membrane electron transport chain. (457 aa) |
| | CMC2 | COX assembly mitochondrial protein 2; Protein involved in respiratory chain complex assembly or maintenance; protein of the mitochondrial intermembrane space; contains twin Cx9C motifs that can form coiled coil-helix-coiled-coil helix fold. (109 aa) |
| | SCO2 | Protein 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) |
| | SCO1 | Copper-binding protein of mitochondrial inner membrane; required for cytochrome c oxidase activity and respiration; may function to deliver copper to cytochrome c oxidase; similar to thioredoxins; SCO1 has a paralog, SCO2, that arose from the whole genome duplication. (295 aa) |
| | COX9 | Subunit VIIa of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain. (59 aa) |
| | MIX14 | Mitochondrial intermembrane space protein of unknown function; required for normal oxygen consumption; contains twin cysteine-x9-cysteine motifs; protein abundance increases in response to DNA replication stress. (121 aa) |
| | PET100 | Protein 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) |
| | COX26 | Cytochrome c oxidase subunit 26, mitochondrial; Stabilizes or regulates formation of respiratory chain supercomplexes composed of Complex III (ubiquinol-cytochrome c reductase) and Complex IV (cytochrome c oxidase). (66 aa) |
| | HIM1 | Protein of unknown function involved in DNA repair. (414 aa) |
| | SDH6 | Mitochondrial 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) |
| | MZM1 | Mitochondrial 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) |
| | SDH7 | Mitochondrial 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) |
| | 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) |
| | RIP1 | Ubiquinol-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) |
| | CYC7 | Cytochrome 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) |
| | FMP52 | Protein FMP52, mitochondrial; Protein of unknown function; localized to the mitochondrial outer membrane; induced by treatment with 8-methoxypsoralen and UVA irradiation; Belongs to the FMP52 family. (231 aa) |
| | PET117 | Protein PET117, mitochondrial; Protein required for assembly of cytochrome c oxidase; Belongs to the PET117 family. (107 aa) |
| | COX15 | Cytochrome 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) |
| | QCR6 | Subunit 6 of the ubiquinol cytochrome-c reductase complex; the complex, also known as the cytochrome bc(1) complex or Complex III, is a component of the mitochondrial inner membrane electron transport chain; highly acidic protein; required for maturation of cytochrome c1; may be loosely associated with the complex since it is easily released into the intermembrane space; Belongs to the UQCRH/QCR6 family. (147 aa) |
| | COX4 | Subunit IV of cytochrome c oxidase; the terminal member of the mitochondrial inner membrane electron transport chain; precursor N-terminal 25 residues are cleaved during mitochondrial import; phosphorylated; spermidine enhances translation. (155 aa) |
| | COX13 | Subunit VIa of cytochrome c oxidase; present in a subclass of cytochrome c oxidase complexes that may have a role in mimimizing generation of reactive oxygen species; not essential for cytochrome c oxidase activity but may modulate activity in response to ATP; required for assembly of Rcf2p into cytochrome c oxidase - cytochrome bc1 supercomplexes. (129 aa) |
| | MTC3 | Maintenance of telomere capping protein 3, mitochondrial; Protein of unknown function; green fluorescent protein (GFP)-fusion protein localizes to the mitochondrion; mtc3 is synthetically sick with cdc13-1. (123 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) |
| | QCR9 | Subunit 9 of ubiquinol cytochrome-c reductase (Complex III); Complex III is a component of the mitochondrial inner membrane electron transport chain; required for electron transfer at the ubiquinol oxidase site of the complex; Belongs to the UQCR10/QCR9 family. (66 aa) |
| | QCR10 | Subunit of the ubiqunol-cytochrome c oxidoreductase complex; this complex comprises part of the mitochondrial respiratory chain; members include Cobp, Rip1p, Cyt1p, Cor1p, Qcr2p, Qcr6p, Qcr7p, Qcr8p, Qcr9p, and Qcr10p and comprises part of the mitochondrial respiratory chain; Belongs to the UQCR11/QCR10 family. (77 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) |
| | CTM1 | Cytochrome c lysine methyltransferase; trimethylates residue 72 of apo-cytochrome c (Cyc1p) in the cytosol; not required for normal respiratory growth. (585 aa) |
| | COX23 | Cytochrome 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) |
| | COX5B | Subunit Vb of cytochrome c oxidase; cytochrome c oxidase is the terminal member of the mitochondrial inner membrane electron transport chain; Cox5Bp is predominantly expressed during anaerobic growth while its isoform Va (Cox5Ap) is expressed during aerobic growth; COX5B has a paralog, COX5A, that arose from the whole genome duplication. (150 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) |
| | PET191 | Mitochondrial protein PET191; Protein required for assembly of cytochrome c oxidase; exists as an oligomer; described as both an integral mitochondrial inner membrane protein facing the intermembrane space (IMS) and as a soluble IMS protein; contains a twin Cx9C motif; imported into the IMS via the MIA import machinery. (108 aa) |
| | CYC1 | Cytochrome c, isoform 1; also known as iso-1-cytochrome c; electron carrier of mitochondrial intermembrane space that transfers electrons from ubiquinone-cytochrome c oxidoreductase to cytochrome c oxidase during cellular respiration; CYC1 has a paralog, CYC7, that arose from the whole genome duplication; human homolog CYC1 can complement yeast null mutant; mutations in human CYC1 cause insulin-responsive hyperglycemia. (109 aa) |
| | CYT2 | Cytochrome c1 heme lyase; involved in maturation of cytochrome c1, which is a subunit of the mitochondrial ubiquinol-cytochrome-c reductase; links heme covalently to apocytochrome c1; human homolog HCCS can complement yeast cyt2 null mutant. (224 aa) |
| | CMC1 | Copper-binding protein of the mitochondrial intermembrane space; evolutionarily conserved; may be involved in delivering copper from the matrix to the cytochrome c oxidase complex; contains a twin CX9C motif. (111 aa) |
| | CCP1 | Mitochondrial cytochrome-c peroxidase; degrades reactive oxygen species in mitochondria, involved in the response to oxidative stress. (361 aa) |
| | COX17 | Copper 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) |
| | COX19 | Protein required for cytochrome c oxidase assembly; located in the cytosol and mitochondrial intermembrane space; putative copper metallochaperone that delivers copper to cytochrome c oxidase; contains twin cysteine-x9-cysteine motifs. (98 aa) |
| | COX12 | Subunit VIb of cytochrome c oxidase; cytochrome c oxidase is also known as respiratory Complex IV and is the terminal member of the mitochondrial inner membrane electron transport chain; required for assembly of cytochrome c oxidase but not required for activity after assembly; phosphorylated; easily released from the intermembrane space, suggesting a loose association with Complex IV. (83 aa) |
| | COA4 | Cytochrome oxidase assembly factor 4; Twin Cx(9)C protein involved in cytochrome c oxidase organization; organization includes assembly or stability; localizes to the mitochondrial intermembrane space via the Mia40p-Erv1p system; interacts genetically with CYC1 and with cytochrome c oxidase assembly factors; Belongs to the COA4 family. (150 aa) |
| | COX8 | Subunit VIII of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain. (78 aa) |
| | RCF1 | Respiratory supercomplex factor 1, mitochondrial; Cytochrome c oxidase subunit; required for assembly of the Complex III-Complex IV supercomplex, and for assembly of Cox13p and Rcf2p into cytochrome c oxidase; similar to Rcf2p, and either Rcf1p or Rcf2p is required for late-stage assembly of the Cox12p and Cox13p subunits and for cytochrome c oxidase activity; required for growth under hypoxic conditions; member of the hypoxia induced gene family; C. elegans and human orthologs are functional in yeast. (159 aa) |
| | MIX17 | Mitochondrial intermembrane space protein; required for normal oxygen consumption; contains twin cysteine-x9-cysteine motifs; protein abundance increases in response to DNA replication stress. (156 aa) |
| | BUD22 | Bud site selection protein 22; Protein required for rRNA maturation and ribosomal subunit biogenesis; required for 18S rRNA maturation; also required for small ribosomal subunit biogenesis; cosediments with pre-ribosomal particles; mutation decreases efficiency of +1 Ty1 frameshifting and transposition, and affects budding pattern. (519 aa) |
| | COA6 | Protein 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) |
| | COX7 | Subunit VII of cytochrome c oxidase (Complex IV); Complex IV is the terminal member of the mitochondrial inner membrane electron transport chain. (60 aa) |
| | COX5A | Subunit Va of cytochrome c oxidase; cytochrome c oxidase is the terminal member of the mitochondrial inner membrane electron transport chain; Cox5Ap is predominantly expressed during aerobic growth while its isoform Vb (Cox5Bp) is expressed during anaerobic growth; COX5A has a paralog, COX5B, that arose from the whole genome duplication. (153 aa) |
| | SAL1 | Truncated non-functional calcium-binding mitochondrial carrier SAL1-1; ADP/ATP transporter; member of the Ca2+-binding subfamily of mitochondrial carriers, with two EF-hand motifs; transport activity of either Sal1p or Pet9p is critical for viability; polymorphic in different S. cerevisiae strains. (494 aa) |
| | RCF2 | Respiratory supercomplex factor 2, mitochondrial; Cytochrome c oxidase subunit; has a role in assembly of respiratory supercomplexes; similar to Rcf1p, and either Rcf1p or Rcf2p is required for late-stage assembly of the Cox12p and Cox13p subunits and for cytochrome c oxidase activity; associates with the cytochrome c oxidase - cytochrome bc1 supercomplex; null mutant accumulates reactive oxygen species; member of the conserved hypoxia induced gene family; C. elegans homolog is functional in yeast. (224 aa) |
| | SDH5 | Succinate 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) |
| | CYC2 | Mitochondrial peripheral inner membrane protein; contains a FAD cofactor in a domain exposed in the intermembrane space; exhibits redox activity in vitro; likely participates in ligation of heme to acytochromes c and c1 (Cyc1p and Cyt1p). (366 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) |
| | COX11 | Cytochrome 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) |
| | COX10 | Protoheme 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) |
| | QCR2 | Subunit 2 of ubiquinol cytochrome-c reductase (Complex III); Complex III is a component of the mitochondrial inner membrane electron transport chain; phosphorylated; transcription is regulated by Hap1p, Hap2p/Hap3p, and heme. (368 aa) |
