NDUFS6 Antibody – Goat Polyclonal

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.[provided by RefSeq, Oct 2009]

NDUFS6: Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in 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. Belongs to the complex I NDUFS6 subunit family.

Protein type: EC 1.6.99.3; Energy Metabolism - oxidative phosphorylation; Mitochondrial; EC 1.6.5.3; Oxidoreductase

Chromosomal Location of Human Ortholog: 5p15.33

Cellular Component: mitochondrial inner membrane; mitochondrial respiratory chain complex I

Molecular Function: electron carrier activity; NADH dehydrogenase (ubiquinone) activity

Biological Process: fatty acid metabolic process; mitochondrial electron transport, NADH to ubiquinone; mitochondrial respiratory chain complex I assembly; multicellular organism growth; multicellular organismal aging; muscle contraction

Disease: Mitochondrial Complex I Deficiency