Coq9
entrez
67914
canonical
uniprot
Q8K1Z0
canonical
ensembl
ENSMUSG00000031782
resolver
ensp
ENSMUSP00000034234
resolver
enst
ENSMUST00000034234
resolver
genesymbol
Coq9
resolver
uniprot
Q9CT61
resolver
uniprot_entry
COQ9_MOUSE
resolver
uniprot_sec
Q9CT61
resolver
amino_acid_sequence
MAATAAVSGVLGRLGWRLLQLRCLPVARCRPALVPRAFHTAVGFRSSEEQKQQPPHSSSQQHSETQGPEFSRPPPRYTDQSGEEEEDYESEEQLQHRILTAALEFVPAHGWTAEAIAEGAQSLGLSSAAASMFGSDGSELILHFVTQCNARLNQVLEEEQKLVQLGQAEKRKTDQFLRDAVETRLRMLIPYIEHWPRALSILLLPHNIPPSLNLLTSMVDDMWHYAGDQSTDFNWYTRRAVLAGIYNTTELVMMQDSSPDFEDTWRFLENRINDAMNMGHTAKQVKSTGEALVQGLMGAAVTLKNLTGLNQRR
uniprot
proteins
function
FUNCTION: Membrane-associated protein that warps the membrane surface to access and bind aromatic isoprenes with high specificity, including ubiquinone (CoQ) isoprene intermediates and presents them directly to COQ7, therefore facilitating the COQ7-mediated hydroxylase step (By similarity). Participates in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration (PubMed:23255162, PubMed:25339443). {ECO:0000250|UniProtKB:O75208, ECO:0000269|PubMed:23255162, ECO:0000269|PubMed:25339443}.
uniprot
proteins
mass_dalton
35083
uniprot
proteins
mutagenesis
MUTAGEN 239..313; /note="Missing: Severely reduces COQ7 protein expression. Severely reduces NADPH-dependent 3-demethoxyubiquinone 3-hydroxylase activity of COQ7. Mutant mice exhibit encephalomyopathy due to a widespread coenzyme Q deficiency and accumulation of demethoxyubiquinone. Lethality between 3 and 6 months of age due to neuronal death and demyelinization with severe vacuolization and astrogliosis in the brain."; /evidence="ECO:0000269|PubMed:23255162, ECO:0000269|PubMed:25339443
uniprot
proteins
pathway_participation
PATHWAY: Cofactor biosynthesis; ubiquinone biosynthesis. {ECO:0000269|PubMed:23255162, ECO:0000269|PubMed:25339443}.
uniprot
proteins
post_translational_modification
PTM: In response to mitochondrial stress, the precursor protein is ubiquitinated by the SIFI complex in the cytoplasm before mitochondrial import, leading to its degradation. Within the SIFI complex, UBR4 initiates ubiquitin chain that are further elongated or branched by KCMF1. {ECO:0000250|UniProtKB:O75208}.
uniprot
proteins
protein_family
COQ9 family
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16141072
uniprot
proteins
pubmed
17208939
uniprot
proteins
pubmed
17242355
uniprot
proteins
pubmed
18614015
uniprot
proteins
pubmed
21183079
uniprot
proteins
pubmed
23255162
uniprot
proteins
pubmed
23576753
uniprot
proteins
pubmed
25339443
uniprot
proteins
sequence_length
313
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion {ECO:0000269|PubMed:18614015}. Note=Associates with cardiolipin-rich membranes which leads to the lipid bilayer deformation and then accessing to membrane-bound lipids. {ECO:0000250|UniProtKB:O75208}.
uniprot
proteins