NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial (NDUFS8)

NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial (NDUFS8) is a **core subunit of mitochondrial complex I (NADH:ubiquinone oxidoreductase)**, crucial for the first and largest enzyme complex of the electron transport chain within the inner mitochondrial membrane[1][2][3]. It is responsible for the transfer of electrons from NADH to ubiquinone (coenzyme Q), playing an essential role in **cellular ATP production through oxidative phosphorylation** and in maintaining the mitochondrial membrane potential[1][2][3]. NDUFS8 contains iron-sulfur clusters essential for electron transfer. Mutations in NDUFS8 impair complex I function, resulting in **severe mitochondrial diseases**, including Leigh syndrome and other neurodegenerative and metabolic disorders[1][3]. Complex I is a known pharmacological target for metabolic modulation (e.g., metformin) and toxicologic inhibition (e.g., rotenone). Dysfunction, whether genetic or pharmacologic, can lead to compromised energy production and heightened production of reactive oxygen species, impacting diverse cellular processes and often manifesting as neuromuscular, cardiac, or metabolic disease[1][2][3].

Other names

NADH:ubiquinone oxidoreductase core subunit S8CI-23kDTYKYCI-23kComplex I-23kDNADH-ubiquinone oxidoreductase 23 kDa subunitTYKY subunitcomplex I 23kDa subunitNADH dehydrogenase (ubiquinone) Fe-S protein 8, 23kDa (NADH-coenzyme Q reductase)NADH-ubiquinone oxidoreductase 23 KDa subunitComplex I 23kDa SubunitMC1DN2CI23KD

Inhibition of electron transfer from NADH to ubiquinone, leading to reduced ATP production and increased reactive oxygen species (for inhibitors like rotenone, metformin); Disruption of mitochondrial membrane potential (as a secondary effect of inhibition)