Recombinant Human 5-Demethoxyubiquinone Hydroxylase, Mitochondrial/COQ7 (C-6His)

Ubiquinone Biosynthesis Protein COQ7 Homolog (COQ7) is a mitochondrion inner membrane and peripheral membrane protein which belongs to the COQ7 family. It is expressed dominantly in heart and skeletal muscle. COQ7 is synthesized as a preprotein that is imported into the mitochondrial matrix, where the sequence is cleaved off and the mature protein becomes loosely associated with the inner membrane. COQ7 is involved in lifespan determination in ubiquinone-independent manner and also involved in ubiquinone biosynthesis. COQ7 is potential central metabolic regulator. Human COQ7 protein contains 179 amino acids, is mostly helical, and contains an alpha-helical membrane insertion. It has been shown that mutations in the gene are associated with increased life span. Defects of the gene slow down a variety of developmental and physiological processes, including the cell cycle, embryogenesis, post-embryonic growth, rhythmic behaviors and aging.