NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 (NDUFB9)

NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 (NDUFB9) is an accessory subunit of mitochondrial complex I (NADH:ubiquinone oxidoreductase), the largest enzyme complex of the electron transport chain, embedded in the inner mitochondrial membrane. NDUFB9 is not directly involved in electron transfer catalysis but is necessary for structural integrity and function of complex I, facilitating the transfer of electrons from NADH to ubiquinone, which is key for ATP synthesis via oxidative phosphorylation. Mutations or reductions in NDUFB9 are linked to mitochondrial complex I deficiency, associated with a spectrum of disorders including neurological, muscular, and metabolic diseases as well as certain cancers. The gene product is a 22kDa protein of the LYRM family, characterized by a conserved LYR motif[1][2][3][4][10][12].

Other names

LYRM3UQOR22CI-B22B22Complex I B22 subunitLYR motif-containing protein 3MC1DN24NDUB9NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 9[1][3][5][10]

Inhibition of complex I disrupts electron flow and ATP production; drugs targeting complex I generally impair mitochondrial function[1][3]