NADH-ubiquinone oxidoreductase chain 1 (ND1)

NADH-ubiquinone oxidoreductase chain 1 (ND1) is a core, mitochondrially encoded subunit of Complex I (NADH:ubiquinone oxidoreductase), the largest enzyme complex in the mitochondrial electron transport chain[5][7][8]. ND1 is essential for the catalytic function of Complex I, which transfers electrons from NADH to ubiquinone (coenzyme Q10), coupling this process to the active translocation of protons across the mitochondrial inner membrane[1][2][3]. This generates an electrochemical gradient used for ATP synthesis by ATP synthase. ND1 forms part of the membrane arm that anchors Complex I in the inner membrane and participates directly in proton translocation and electron transfer. Defects or mutations in ND1 are linked to mitochondrial diseases and impaired energy metabolism, contributing to disorders such as Leber hereditary optic neuropathy and other neuromuscular syndromes[4]. Complex I is also a pharmacological target for several natural and synthetic inhibitors, including rotenone and piericidin, which disrupt energy metabolism and are used experimentally to study mitochondrial dysfunction and its consequences[1][4].

Other names

ND1NADH dehydrogenase subunit 1Complex I chain 1

Inhibition of electron transfer from NADH to ubiquinone, disrupting ATP production and increasing oxidative stress