COQ4 : coenzyme Q4
Description
The COQ4 (coenzyme Q4) is a protein-coding gene located on chromosome 9.
The COQ4 gene provides instructions for making a protein involved in the production of coenzyme Q10, although its exact role is unknown. Research suggests the COQ4 protein helps organize other proteins involved in coenzyme Q10 production into a stable functional group (a protein complex). Coenzyme Q10 has several critical functions in cells. It plays a vital role in mitochondria during oxidative phosphorylation, converting food energy into usable energy. Coenzyme Q10 is also involved in producing pyrimidines (building blocks of DNA and RNA) and molecules like ATP and GTP (energy sources in the cell). In cell membranes, coenzyme Q10 acts as an antioxidant, protecting cells from damage caused by free radicals, a byproduct of energy production.
COQ4 is also known as CGI-92, COQ10D7, SPAX10.
Associated Diseases
- Coenzyme Q10 deficiency, primary, 7
- Spastic ataxia 10, autosomal recessive
- Primary coenzyme Q10 deficiency