Entrez Summary: This locus represents a mitochondrial ubiquinone biosynthesis gene. The encoded protein is likely necessary for biosynthesis of coenzyme Q10, as mutations at this locus have been associated with autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency.[provided by RefSeq, Sep 2010].
UniProt Summary: Lipid-binding protein involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration. Binds a phospholipid of at least 10 carbons in each acyl group. May be required to present its bound-lipid to COQ7. {ECO:0000269|PubMed:25339443}.
Pfam DomainsGO Terms
Pfam Domains
COQ9
GO Terms
ubiquinone biosynthetic process
quinone biosynthetic process
ubiquinone metabolic process
ketone biosynthetic process
quinone metabolic process
mitochondrial electron transport, NADH to ubiquinone
oxidoreduction coenzyme metabolic process
cellular ketone metabolic process
mitochondrial ATP synthesis coupled electron transport
ATP synthesis coupled electron transport
respiratory electron transport chain
oxidative phosphorylation
coenzyme biosynthetic process
lipid binding
cellular respiration
electron transport chain
ATP metabolic process
cofactor biosynthetic process
energy derivation by oxidation of organic compounds
coenzyme metabolic process
mitochondrial inner membrane
generation of precursor metabolites and energy
cofactor metabolic process
small molecule biosynthetic process
protein homodimerization activity
oxidation-reduction process
mitochondrion
phosphorylation
small molecule metabolic process
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)