COQ9
Description
The COQ9 (coenzyme Q9) is a protein-coding gene located on chromosome 16.
COQ9 is a gene that encodes a mitochondrial protein involved in the biosynthesis of coenzyme Q10. Mutations in this gene can lead to autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency. Coenzyme Q10 deficiency can cause a range of health problems, including muscle weakness, developmental delays, and heart problems.
COQ9 is a lipid-binding protein that plays a crucial role in the biosynthesis of coenzyme Q, also known as ubiquinone. Coenzyme Q is an essential lipid-soluble electron transporter required for aerobic cellular respiration. COQ9 binds to phospholipids with at least 10 carbons in each acyl group and may be involved in presenting these lipids to the COQ7 protein.
COQ9 is also known as C16orf49, COQ10D5.
