Recombinant Human COQ7 (C-6His)

Source : Human Cells,Ubiquinone Biosynthesis Protein COQ7 Homolog is a mitochondrion inner membrane and peripheral membrane protein that belongs to the COQ7 family. It is expressed primarily in heart and skeletal muscle. COQ7 is synthesized as a preprotein that is transmitted into the mitochondrial matrix, where the sequence is cleaved off and the mature protein becomes loosely associated with the inner membrane. COQ7 plays a role in lifespan determination in ubiquinone-independent manner, as well as ubiquinone biosynthesis. COQ7 also acts as a potential central metabolic regulator. 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. Protein function: Catalyzes the hydroxylation of 2-polyprenyl-3-methyl-6- methoxy-1,4-benzoquinol (DMQH2) during ubiquinone biosynthesis. Has also a structural role in the COQ enzyme complex, stabilizing other COQ polypeptides. Involved in lifespan determination in a ubiquinone-independent manner. Plays a role in modulating mitochondrial stress responses, acting in the nucleus, perhaps via regulating gene expression, independent of its characterized mitochondrial function in ubiquinone biosynthesis (PubMed:25961505). [The UniProt Consortium]