coenzyme Q4

Normal function

Der COQ4 gene provides instructions for making a protein that is involved in the production of a molecule called coenzyme Q10, although its specific role in this process is unknown. Research suggests that the COQ4 protein may help organize other proteins involved in coenzyme Q10 production into a stable functional group (a protein complex).

Coenzyme Q10 has several critical functions in cells throughout the body. In cell structures called mitochondria, coenzyme Q10 plays an essential role in a process called oxidative phosphorylation, which converts the energy from food into a form cells can use. Coenzyme Q10 is also involved in producing pyrimidines, which are building blocks of DNA, its chemical cousin RNA, and molecules such as ATP and GTP that serve as energy sources in the cell. In cell membranes, coenzyme Q10 acts as an antioxidant, protecting cells from damage caused by unstable oxygen-containing molecules (free radicals), which are byproducts of energy production.

Conditions referring to this gene or chromosome

• Primary coenzyme Q10 deficiency

Other names

CGI-92

coenzyme Q biosynthesis protein 4 homolog

coenzyme Q4 homolog

COQ10D7

ubiquinone biosynthesis protein COQ4 homolog, mitochondrial isoform 1 precursor

ubiquinone biosynthesis protein COQ4 homolog, mitochondrial isoform 2 precursor