Mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 1 (MT-ND1)

Mitochondrially encoded NADH:ubiquinone oxidoreductase core subunit 1 (MT-ND1) is a protein encoded by the mitochondrial genome that forms an integral membrane component of complex I (NADH:ubiquinone oxidoreductase) of the mitochondrial electron transport chain[1][3][5]. As a core subunit, it is directly involved in the catalytic processes of electron transfer from NADH to ubiquinone (coenzyme Q10) and the concomitant pumping of protons across the inner mitochondrial membrane, which is essential for the generation of the proton gradient used to synthesize ATP[1][3]. Mutations in the MT-ND1 gene are associated with several mitochondrial diseases including MELAS, Leigh syndrome, and Leber's hereditary optic neuropathy, highlighting its critical role in cellular energy metabolism and human disease[1][3][5]. Drugs inhibiting complex I can act through MT-ND1, leading to mitochondrial dysfunction often leveraged experimentally and potentially relevant pharmacologically.

Other names

NADH-ubiquinone oxidoreductase chain 1MT-ND1MTND1NADH1ND1NAD1NADH dehydrogenase subunit 1complex I ND1 subunit

Inhibition of electron transport (e.g., by rotenone, metformin), Disruption of proton gradient and ATP synthesis