NADH Dehydrogenase Ubiquinone Fe-S Protein 8 (NDUFS8)

NDUFS8 encodes a subunit of mitochondrial NADH:ubiquinone oxidoreductase, or Complex I, a multimeric enzyme of the respiratory chain responsible for NADH oxidation, ubiquinone reduction, and the ejection of protons from mitochondria. The encoded protein is involved in the binding of two of the six to eight iron-sulfur clusters of Complex I and, as such, is required in the electron transfer process. Mutations in this gene have been associated with Leigh syndrome.

The NDUFS8 subunit of complex I is highly conserved among eukaryotes and prokaryotes and contains 2 4Fe4S ferredoxin consensus patterns, which appear to provide the binding sites for the iron-sulfur cluster N-2. The NDUFS8 cDNA encodes a predicted 210-amino acid protein.