NADH:ubiquinone oxidoreductase complex assembly factor Q (PYURF)

NADH:ubiquinone oxidoreductase complex assembly factor Q (PYURF) is a protein encoded by the PYURF gene, also known as PIGY upstream open reading frame. This protein is produced from a bicistronic transcript that also encodes PIGY, but the two proteins are unrelated. PYURF functions in mitochondria as an S-adenosylmethionine-dependent methyltransferase chaperone crucial for the stabilization and assembly of complex I of the respiratory electron transport chain and for coenzyme Q biosynthesis by stabilizing components such as COQ5 and assembly factors such as NDUFAF5[3]. Defects in the PYURF gene are associated with mitochondrial diseases and intellectual developmental disorders[3]. The protein is involved in mitochondrial localization and protein binding, contributing to protein stabilization and carbohydrate derivative metabolism[5][6][3][1][2].\n \nThere are no known drugs or small-molecule inhibitors directly targeting PYURF as of the current literature. It is not currently considered a direct therapeutic target (e.g., receptor, enzyme, transporter), but its essential role in the biogenesis of mitochondrial respiratory complexes makes it a critical component in mitochondrial physiology[3][1][2].

Other names

PIGY upstream open reading frameProtein preY, mitochondrialNDUFAFQPREYPreYPIGY upstream reading frame proteinprotein preY, mitochondrial