NADH:ubiquinone oxidoreductase subunit A8 (NDUFA8)

NADH:ubiquinone oxidoreductase subunit A8 (NDUFA8) is an **accessory subunit** of mitochondrial Complex I, the first and largest enzyme complex of the electron transport chain. It is **not directly involved in catalysis**, but is essential for structural integrity and proper function of Complex I, which catalyzes electron transfer from NADH to ubiquinone coupled with proton translocation across the mitochondrial inner membrane. This process is crucial for ATP generation by oxidative phosphorylation[5][6][1][4][2]. Defects in Complex I, including its accessory subunits such as NDUFA8, are linked to various mitochondrial diseases and are implicated in some neurodegenerative diseases[1][5].

Other names

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8Complex I-19kD subunitCI-PGIVPGIVMGC793Complex I PGIV subunitNADH-ubiquinone oxidoreductase 19 kDa subunitMC1DN37

Inhibition of electron transfer from NADH to ubiquinone at Complex I (i.e., inhibition of NADH:ubiquinone oxidoreductase impairs mitochondrial respiration)