Q99KD6
uniprot
Q99KD6
canonical
amino_acid_sequence
MYYKFSSFTQKLAGAWASEAYTPQGLKPVSTEAPPIIFATPTKLTSSVTAYDYSGKNKVPELQKFFQKADGVPIHLKRGLPDQMLYRTTMALTLGGTIYCLIALYMASQPRNK
uniprot
proteins
function
FUNCTION: Assembly factor that mediates the formation of some mitochondrial respiratory supercomplexes (respirasomes), thereby promoting oxidative phosphorylation and energy metabolism (PubMed:23812712, PubMed:23857330, PubMed:25470551, PubMed:27545886, PubMed:27775717, PubMed:27997587, PubMed:32637615, PubMed:34616041, PubMed:38575788). Acts as a molecular adapter that associates with both mitochondrial respiratory complexes III (CIII) and IV (CIV), promoting their association (PubMed:27545886, PubMed:27775717, PubMed:32637615, PubMed:34616041, PubMed:38575788). Mediates the formation of various mitochondrial respiratory supercomplexes, such as MCIII(2)IV(2), composed of two CIII and two CIV, and the CS-respirasome (MCI(1)III(2)IV(2)), composed of one CI, two CIII and two CIV (PubMed:27775717, PubMed:32637615, PubMed:34616041, PubMed:38575788). Not involved in the formation of the canonical respirasome (MCI(1)III(2)IV(1)), composed of one CI, two CIII and one CIV (PubMed:38575788). The formation of different respirasomes is important for cell adaptation to oxygen conditions and prevent metabolic exhaustion: supercomplexes mediated by COX7A2L/SCAF1 are required to maintain oxidative phosphorylation upon low oxygen conditions and promote metabolic rewiring toward glycolysis (PubMed:38575788). {ECO:0000269|PubMed:23812712, ECO:0000269|PubMed:23857330, ECO:0000269|PubMed:25470551, ECO:0000269|PubMed:27545886, ECO:0000269|PubMed:27775717, ECO:0000269|PubMed:27997587, ECO:0000269|PubMed:32637615, ECO:0000269|PubMed:34616041, ECO:0000269|PubMed:38575788}.
uniprot
proteins
mass_dalton
12562
uniprot
proteins
mutagenesis
MUTAGEN 75; /note="H->A: Abolished ability to promote association between both mitochondrial respiratory complexes III (CIII) and IV (CIV)."; /evidence="ECO:0000269|PubMed:27775717
uniprot
proteins
pathway_participation
PATHWAY: Energy metabolism; oxidative phosphorylation. {ECO:0000250|UniProtKB:P56392}.
uniprot
proteins
protein_family
Cytochrome c oxidase VIIa family
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
23812712
uniprot
proteins
pubmed
23857330
uniprot
proteins
pubmed
25470551
uniprot
proteins
pubmed
27545886
uniprot
proteins
pubmed
27775717
uniprot
proteins
pubmed
27997587
uniprot
proteins
pubmed
31023583
uniprot
proteins
pubmed
32637615
uniprot
proteins
pubmed
34616041
uniprot
proteins
pubmed
36253618
uniprot
proteins
pubmed
38575788
uniprot
proteins
sequence_length
113
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000269|PubMed:27775717, ECO:0000269|PubMed:34616041, ECO:0000269|PubMed:38575788}; Single-pass membrane protein {ECO:0000269|PubMed:34616041, ECO:0000269|PubMed:38575788}.
uniprot
proteins
transmembrane_region
TRANSMEM 81..106; /note="Helical"; /evidence="ECO:0000269|PubMed:34616041, ECO:0000269|PubMed:38575788, ECO:0007744|PDB:7O37, ECO:0007744|PDB:7O3C, ECO:0007744|PDB:8PW5
uniprot
proteins