NADH:ubiquinone oxidoreductase complex assembly factor 3 (NDUFAF3)

NADH:ubiquinone oxidoreductase complex assembly factor 3 (NDUFAF3) is a mitochondrial protein essential for the assembly of complex I (NADH:ubiquinone oxidoreductase) in the electron transport chain[1][2][3][5][6]. It interacts with complex I subunits and is required for the biogenesis of this large enzymatic complex, enabling proper cellular respiration and energy production[1][2]. Mutations in NDUFAF3 can cause severe mitochondrial complex I deficiency, often presenting as fatal neonatal metabolic disorders or Leigh syndrome, highlighting its critical role in mitochondrial bioenergetics[2]. As an assembly factor, it does not directly act as an enzyme or druggable target, but dysfunction of this protein contributes significantly to mitochondrial diseases[1][2][4]. NDUFAF3 acts in concert with other assembly factors, such as NDUFAF4, during early steps of complex I formation[2].

Other names

C3orf60MGC10527DKFZP564J0123E3-32P1MC1DN18NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3nuclear protein E3-3