NADH Dehydrogenase [Ubiquinone] 1 Beta Subcomplex Subunit 3 (NDUFB3) Human Recombinant Protein

This gene encodes an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which is the first enzyme in the electron transport chain of mitochondria. This protein localizes to the inner membrane of the mitochondrion as a single-pass membrane protein. Mutations in this gene contribute to mitochondrial complex 1 deficiency. Alternative splicing results in multiple transcript variants encoding the same protein. Humans have multiple pseudogenes of this gene. [provided by RefSeq, Mar 2012]

NDUFB3: 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 NDUFB3 subunit family.

Protein type: Oxidoreductase; Membrane protein, integral; EC 1.6.5.3; Mitochondrial; Energy Metabolism - oxidative phosphorylation; EC 1.6.99.3

Chromosomal Location of Human Ortholog: 2q31.3

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

Molecular Function: NADH dehydrogenase (ubiquinone) activity

Biological Process: cellular metabolic process; mitochondrial electron transport, NADH to ubiquinone

Disease: Mitochondrial Complex I Deficiency