NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 (NDUFS5)

NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 (NDUFS5) is a small accessory subunit of mitochondrial complex I, the first enzyme complex of the respiratory electron transport chain located in the inner mitochondrial membrane[1][8]. NDUFS5 is not directly involved in catalysis but is required for the correct assembly and stability of complex I. Complex I catalyzes the transfer of electrons from NADH to ubiquinone, coupling this process to proton translocation and ATP synthesis in oxidative phosphorylation[1][2][8]. Mutations or deficiencies in NDUFS5 are associated with mitochondrial complex I deficiency, manifesting as diverse mitochondrial disorders including progressive leukoencephalopathy and ovarian failure[1][7].

Other names

NADH:ubiquinone oxidoreductase subunit S5CI-15 kDaCI-15kComplex I-15 kDaNADH-ubiquinone oxidoreductase 15 kDa subunitcomplex I 51kDa subunitNADH dehydrogenase (ubiquinone) Fe-S protein 5, 15kDa (NADH-coenzyme Q reductase)NADH:ubiquinone oxidoreductase 15 kDa IP subunitNDUFS5

Inhibitors of complex I block electron transfer from NADH to ubiquinone, leading to impaired mitochondrial respiration and ATP synthesis, but these do not specifically select the NDUFS5 subunit[8].