NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 (NDUFA6)

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 (NDUFA6) is an accessory subunit of mitochondrial complex I, the first and largest enzyme complex of the oxidative phosphorylation respiratory chain. It belongs to the LYR family, characterized by a conserved LYR tripeptide motif near the N-terminus and is required for proper assembly and function of complex I but is not involved directly in the catalytic electron transfer. Defects in NDUFA6 are associated with certain forms of mitochondrial disease, notably nuclear type mitochondrial complex I deficiency 33. Complex I transfers electrons from NADH to ubiquinone, driving oxidative phosphorylation for ATP production and cellular energy metabolism. NDUFA6 is not a receptor or transporter, but an enzyme accessory protein essential for mitochondrial energy production. Current therapeutics do not directly target NDUFA6 individually, but overall complex I modulating drugs may affect its function indirectly.

Other names

LYRM6NADHB14CI-B14B14Complex I-B14LYR motif-containing protein 6NADH-ubiquinone oxidoreductase B14 subunitMC1DN33

Inhibitors of complex I (such as rotenone) block the electron transfer from NADH to ubiquinone in the respiratory chain. Modulating complex I function affects cellular respiration and ATP generation, but accessory subunit targeting is not a standard therapeutic approach.