Entrez Summary: This gene encodes a mitochondrial protein similar to yeast ABC1, which functions in an electron-transferring membrane protein complex in the respiratory chain. It is not related to the family of ABC transporter proteins. Expression of this gene is induced by the tumor suppressor p53 and in response to DNA damage, and inhibiting its expression partially suppresses p53-induced apoptosis. Alternatively spliced transcript variants have been found; however, their full-length nature has not been determined. [provided by RefSeq, Jul 2008].
UniProt Summary: Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration (PubMed:25498144, PubMed:21296186, PubMed:25540914, PubMed:27499294). Its substrate specificity is unclear: does not show any protein kinase activity (PubMed:25498144, PubMed:27499294). Probably acts as a small molecule kinase, possibly a lipid kinase that phosphorylates a prenyl lipid in the ubiquinone biosynthesis pathway, as suggested by its ability to bind coenzyme Q lipid intermediates (PubMed:25498144, PubMed:27499294). Shows an unusual selectivity for binding ADP over ATP (PubMed:25498144). {ECO:0000269|PubMed:25498144, ECO:0000269|PubMed:27499294, ECO:0000305|PubMed:21296186, ECO:0000305|PubMed:25540914}.
Pfam DomainsGO Terms
Pfam Domains
ABC1
GO Terms
extrinsic component of mitochondrial inner membrane
ubiquinone biosynthetic process
quinone biosynthetic process
ubiquinone metabolic process
ketone biosynthetic process
quinone metabolic process
ADP binding
oxidoreduction coenzyme metabolic process
cellular ketone metabolic process
kinase activity
coenzyme biosynthetic process
cofactor biosynthetic process
protein kinase activity
coenzyme metabolic process
cofactor metabolic process
small molecule biosynthetic process
protein phosphorylation
mitochondrion
phosphorylation
ATP binding
small molecule metabolic process
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)