O75489

NDUFS3 (NADH dehydrogenase ubiquinone iron-sulfur protein 3) is one of about 45 subunits comprising complex I of the oxidative phosphorylation electron transport chain. The multisubunit NADH: ubiquinone oxidoreductase (complex I) is the first enzyme complex in the electron transport chain of the mitochondria. NDUFS3 is the last subunit of the seven subunits that make up the core of complex I. Through use of chaotropic agents, complex I can be separated into three different fractions: a flavoprotein fraction, an iron-sulfur protein (IP) fraction, and a hydrophobic protein (HP) fraction. The IP fraction includes NDUFS1-7. NDUFS3 contains a highly conserved casein kinase II phosphorylation site. Mutations in the NDUFS3 gene may cause optic atrophy, Leigh syndrome and complex I deficiency.