NADH:ubiquinone oxidoreductase subunit A2 (NDUFA2)

NADH:ubiquinone oxidoreductase subunit A2 (NDUFA2) is an accessory subunit of mitochondrial Complex I, the first and largest enzyme complex of the electron transport chain situated in the mitochondrial inner membrane. The protein consists of 99 amino acids and contributes to the hydrophobic fraction that helps anchor Complex I to the membrane and supports assembly and regulation. While NDUFA2 is not involved in the catalytic process of electron transfer, it is critical for proper complex formation and function. Mutations in the NDUFA2 gene cause mitochondrial Complex I deficiency and Leigh syndrome, a progressive neurodegenerative disorder characterized by early-onset neurological decline. There are no known drugs that selectively target NDUFA2, but pharmacological inhibition of Complex I indirectly affects its function in the electron transport chain.

Other names

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2Complex I B8 subunitB8CI-B8Complex I-B8NADH-ubiquinone oxidoreductase B8 subunitMC1DN13CD14CIB8

Inhibition of electron transfer through mitochondrial Complex I; Disruption of proton pumping and mitochondrial membrane potential via Complex I blockade (generic for Complex I inhibitors like rotenone; not specific for NDUFA2 subunit)