COQ7
Description
The COQ7 (coenzyme Q7, hydroxylase) is a protein-coding gene located on chromosome 16.
The COQ7 gene encodes the mitochondrial enzyme 5-demethoxyubiquinone hydroxylase (DMQ hydroxylase), also known as coenzyme Q7 hydroxylase, which is essential for ubiquinone biosynthesis. This protein is highly conserved across different species, including humans, mice, fruit flies, and yeast. The human COQ7 gene consists of six exons spanning 11 kb and is located on chromosome 16. The encoded protein is approximately 24 kilodaltons and has two repeats of approximately 90 amino acids that are important for iron coordination. Ubiquinone is a vital cofactor in numerous cellular redox processes, particularly in mitochondrial electron transport. It is also an important antioxidant in the cell.
COQ7 catalyzes the hydroxylation of 2-polyprenyl-3-methyl-6-methoxy-1,4-benzoquinol (DMQH2) during ubiquinone biosynthesis. It also plays a structural role in the COQ enzyme complex, stabilizing other COQ polypeptides. COQ7 is involved in lifespan determination, independent of ubiquinone. It modulates mitochondrial stress responses by acting in the nucleus, potentially through gene expression regulation, independent of its known mitochondrial function in ubiquinone biosynthesis.
COQ7 is also known as CAT5, CLK-1, CLK1, COQ10D8, HMNR9.
