Mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 3 (MT-ND3)

Mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 3 (MT-ND3) is a core subunit of respiratory chain complex I, the largest enzyme complex of the mitochondrial electron transport system. MT-ND3 is essential for the catalytic activity of complex I, which transfers electrons from NADH to ubiquinone, contributing to the generation of the proton gradient and ATP synthesis in mitochondria. Variants in MT-ND3 cause inherited mitochondrial diseases such as Leigh syndrome and Leber hereditary optic neuropathy and are implicated in Parkinson's disease. MT-ND3 itself is not a classic drug target, but is crucial in the function and dysfunction of the mitochondrial respiratory chain, and is important for diagnosis and study of mitochondrial diseases.

Other names

NADH-ubiquinone oxidoreductase chain 3MTND3NADH3ND3NAD3NADH dehydrogenase subunit 3complex I ND3 subunit

Inhibition of mitochondrial respiratory chain complex I, disruption of electron transport, and reduction of NADH oxidation can occur with inhibitors of complex I (e.g., rotenone acts by direct inhibition of site within complex I)