Rabbit anti-Human COQ8A Polyclonal Antibody

Uniprot Id

Q8NI60

Target Species

Human

Target Name

COQ8A

Target Full Name

Atypical kinase COQ8A, mitochondrial

Target Function

Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration. Its substrate specificity is unclear: does not show any protein kinase activity. 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. Shows an unusual selectivity for binding ADP over ATP.

Target Involvement

Coenzyme Q10 deficiency, primary, 4 (COQ10D4)

Target Subcellular Location

Mitochondrion. Membrane; Single-pass membrane protein.

Target Protein Families

Protein kinase superfamily, ADCK protein kinase family

Target Tissue Specificity

Widely expressed, with highest levels in adrenal gland, heart, pancreas, nasal mucosa, stomach, uterus and skeletal muscle.

Target Synonyms

aarF domain containing protein kinase 3; aarF domain-containing protein kinase 3; ADCK 3; Adck3; ADCK3_HUMAN; ARCA2; CABC 1; CABC1 ; Chaperone ABC1 (activity of bc1 complex S.pombe) like; Chaperone ABC1 activity of bc1 complex homolog; Chaperone ABC1 like; Chaperone activity of bc1 complex like; Chaperone activity of bc1 complex like mitochondrial; Chaperone activity of bc1 complex-like; Chaperone-ABC1-like; Coenzyme Q8 homolog; COQ 8; COQ10D4; COQ8; COQ8; S. cerevisiae; homolog of; MGC4849; mitochondrial; OTTHUMP00000035734; OTTHUMP00000035737; SCAR9

Target Background

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.