NADH Dehydrogenase [Ubiquinone] Flavoprotein 2 (NDUFV2) Antibody – Rabbit Polyclonal

The NADH-ubiquinone oxidoreductase complex (complex I) of the mitochondrial respiratory chain catalyzes the transfer of electrons from NADH to ubiquinone, and consists of at least 43 subunits. The complex is located in the inner mitochondrial membrane. This gene encodes the 24 kDa subunit of complex I, and is involved in electron transfer. Mutations in this gene are implicated in Parkinson's disease, bipolar disorder, schizophrenia, and have been found in one case of early onset hypertrophic cardiomyopathy and encephalopathy. A non-transcribed pseudogene of this locus is found on chromosome 19. [provided by RefSeq, Oct 2009]

NDUFV2: 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. Belongs to the complex I 24 kDa subunit family.

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

Chromosomal Location of Human Ortholog: 18p11.22

Cellular Component: mitochondrial inner membrane; mitochondrial respiratory chain complex I; mitochondrion; myelin sheath

Molecular Function: 2 iron, 2 sulfur cluster binding; electron carrier activity; metal ion binding; NADH dehydrogenase (ubiquinone) activity

Biological Process: cardiac muscle development; cellular metabolic process; mitochondrial electron transport, NADH to ubiquinone; mitochondrial respiratory chain complex I assembly; nervous system development

Disease: Mitochondrial Complex I Deficiency