This gene 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.
