NADH-Ubiquinone Oxidoreductase Chain 3 (Mtnd3) (ND3) Rat Recombinant Protein

MT-ND3: 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. Defects in MT-ND3 are a cause of Leigh syndrome (LS). LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Defects in MT-ND3 are a cause of mitochondrial complex I deficiency (MT-C1D). A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. Belongs to the complex I subunit 3 family.

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

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

Molecular Function: NADH dehydrogenase (ubiquinone) activity

Biological Process: response to light intensity; response to hormone stimulus; response to oxidative stress