NADH Dehydrogenase Ubiquinone Fe-S Protein 1 (NDUFS1)

NDUFS1 belongs to the complex I 75 kDa subunit family. Mammalian complex I is composed of 45 different subunits. It locates at the mitochondrial inner membrane. This protein has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to the respiratory chain. This protein is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized. Mutations in this gene are associated with complex I deficiency.The predicted 727-amino acid human 75-kD Fe-S protein contains a 23-amino acid presequence and is 97% identical to the bovine homolog. Various cysteine-rich motifs similar to those found in rubredoxins, the Reiske Fe-S protein in Neurospora, and 4 Fe-4S ferredoxins are present in the protein sequence.