This gene encodes one of the iron-sulfur protein (IP) components of mitochondrial NADH:ubiquinone oxidoreductase (complex I). Mutations in this gene are associated with Leigh syndrome resulting from mitochondrial complex I deficiency.[provided by RefSeq, Apr 2009],catalytic activity:NADH + acceptor = NAD(+) + reduced acceptor.,catalytic activity:NADH + ubiquinone = NAD(+) + ubiquinol.,function:Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.,similarity:Belongs to the complex I 30 kDa subunit family.,subunit:Mammalian complex I is composed of 45 different subunits.,
