NADH Dehydrogenase Ubiquinone Fe-S Protein 6 (NDUFS6)

NDUFS6 encodes a subunit of the NADH:ubiquinone oxidoreductase (complex I), which is the first enzyme complex in the electron transport chain of mitochondria. This complex functions in the transfer of electrons from NADH to the respiratory chain. The subunit encoded by this gene is one of seven subunits in the iron-sulfur protein fraction. Mutations in this gene cause mitochondrial complex I deficiency, a disease that causes a wide variety of clinical disorders, including neonatal disease and adult-onset neurodegenerative disorders.The predicted 124-amino acid human NDUFS6 protein shares 83% identity with the bovine 13-kD protein. The first 28 amino acids of human NDUFS6 comprise a possible mitochondrial targeting sequence.