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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) | ||||
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) | ||||
COX20 | Mitochondrial inner membrane protein; required for proteolytic processing of Cox2p and its assembly into cytochrome c oxidase; Belongs to the COX20 family. (205 aa) | ||||
MRPL35 | Mitochondrial ribosomal protein of the large subunit; Belongs to the phosphatidylethanolamine-binding protein family. Mitochondrion-specific ribosomal protein mL38 subfamily. (367 aa) | ||||
YDR381C-A | Protein of unknown function; localized to the mitochondrial outer membrane. (114 aa) | ||||
PET117 | Protein PET117, mitochondrial; Protein required for assembly of cytochrome c oxidase; Belongs to the PET117 family. (107 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) | ||||
FMP32 | Protein FMP32, mitochondrial; Putative assembly factor for cytochrome c oxidase; the authentic, non-tagged protein is detected in highly purified mitochondria in high-throughput studies; has similarity to human MCUR1/CCDC90A. (207 aa) | ||||
COX18 | Cytochrome c oxidase assembly protein COX18, mitochondrial; Protein required for membrane insertion of C-terminus of Cox2p; mitochondrial integral inner membrane protein; interacts genetically and physically with Mss2p and Pnt1p; similar to S. cerevisiae Oxa1, N. crassa Oxa2p, and E. coli YidC; respiratory defect of the null mutant is functionally complemented by human COX18 carrying the N-terminal 54 amino acids of S. cerevisiae Cox18p; Belongs to the OXA1/ALB3/YidC family. (316 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) | ||||
COA1 | Cytochrome c oxidase assembly factor 1; Mitochondrial inner membrane protein; required for assembly of the cytochrome c oxidase complex (complex IV); interacts with complex IV assembly factor Shy1p during the early stages of assembly. (197 aa) | ||||
COX16 | Mitochondrial inner membrane protein; required for assembly of cytochrome c oxidase. (118 aa) | ||||
COA3 | Mitochondrial protein required for cytochrome c oxidase assembly; also involved in translational regulation of Cox1p and prevention of Cox1p aggregation before assembly; located in the mitochondrial inner membrane. (85 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) | ||||
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) | ||||
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) | ||||
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) | ||||
MSS51 | Protein MSS51, mitochondrial; Specific translational activator for the mitochondrial COX1 mRNA; loosely associated with the matrix face of the mitochondrial inner membrane; localizes to vacuole membrane in response to H2O2; influences both COX1 mRNA translation and Cox1p assembly into cytochrome c oxidase; binds to heme B, which may be a mechanism for sensing oxygen levels in order to regulate cytochrome c oxidase biogenesis. (436 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) | ||||
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) | ||||
COX14 | Mitochondrial cytochrome c oxidase (complex IV) assembly factor; also involved in translational regulation of Cox1p and prevention of Cox1p aggregation before assembly; associates with complex IV assembly intermediates and complex III/complex IV supercomplexes; located in the mitochondrial membrane. (70 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) | ||||
PET111 | Protein PET111, mitochondrial; Mitochondrial translational activator specific for the COX2 mRNA; located in the mitochondrial inner membrane. (800 aa) | ||||
MRP7 | Mitochondrial ribosomal protein of the large subunit. (371 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) | ||||
COA2 | Cytochrome oxidase assembly factor; null mutation results in respiratory deficiency with specific loss of cytochrome oxidase activity; functions downstream of assembly factors Mss51p and Coa1p and interacts with assembly factor Shy1p; Belongs to the COA2 family. (68 aa) |