NADH:ubiquinone oxidoreductase subunit B10 (NDUFB10)

NADH:ubiquinone oxidoreductase subunit B10 (NDUFB10) is a protein-coding gene of the mitochondrial respiratory chain complex I, the first and largest enzyme complex in the mitochondrial electron transport chain. NDUFB10 is an accessory subunit, required for the assembly and stability of complex I, which catalyzes electron flow from NADH to ubiquinone, coupled with proton translocation across the inner mitochondrial membrane, ultimately driving ATP synthesis. Mutations or dysfunction in NDUFB10 can lead to mitochondrial complex I deficiency, a group of severe metabolic disorders with neuromuscular and multisystem involvement. NDUFB10 is not a direct drug target but is a subunit of the clinically significant complex I enzyme, which is targeted by inhibitors such as piericidin A and rotenone. Disease involvement includes neurodegenerative and metabolic conditions. Complex I inhibitors pose risks of mitochondrial toxicity and functional impairment

Other names

NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10CI-PDSWPDSWComplex I-PDSWNADH-ubiquinone oxidoreductase PDSW subunitcomplex I PDSW subunitMC1DN35

Competitive inhibition of the ubiquinone-binding site (e.g., piericidin A mimics ubiquinone and blocks electron transfer) Noncompetitive inhibition (other inhibitors may block electron transfer or alter conformation)