NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial (NDUFS4)

NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial (NDUFS4), is a nuclear-encoded accessory subunit of mitochondrial respiratory chain complex I (NADH:ubiquinone oxidoreductase). It is a peripheral membrane protein located on the matrix side of the inner mitochondrial membrane and contributes to electron transport from NADH to ubiquinone, facilitating ATP synthesis via oxidative phosphorylation. Although NDUFS4 is not directly involved in catalysis, it is essential for proper assembly and stabilization of complex I and may be regulated by phosphorylation. Mutations cause mitochondrial complex I deficiency, Leigh syndrome, and are associated with a broad spectrum of multi-organ mitochondrial diseases, especially neurological and muscular phenotypes; these disorders are often severe and clinically heterogeneous.

Other names

AQDQCI-18CI-18 kDaCI-AQDQMC1DN1Complex I-18 kDa subunitNADH-ubiquinone oxidoreductase 18 kDa subunitcomplex I 18kDa subunitCI-18 kDa subunitNADH dehydrogenase (ubiquinone) Fe-S protein 418kDa subunit (NADH-coenzyme Q reductase)mitochondrial respiratory chain complex I (18-KD subunit)

Direct inhibition of complex I (e.g., by rotenone, reducing electron transport and ATP production)