NADH dehydrogenase [ubiquinone] iron-sulfur protein chain (NDUFS (generic), or NDUFS1, NDUFS4, NDUFS6, NDUFS8, etc. for specific subunits)

NADH dehydrogenase [ubiquinone] iron-sulfur proteins comprise a series of essential subunits within mitochondrial Complex I, the first enzyme complex of the electron transport chain. These proteins catalyze electron transfer from NADH to ubiquinone via iron-sulfur clusters embedded in the protein subunits, contributing to the generation of the proton gradient used for ATP synthesis. Several specific subunits exist (e.g., NDUFS1, NDUFS4, NDUFS6, NDUFS8), each encoded by different nuclear genes and responsible for electron transfer or Complex I assembly. Mutations in these subunits are associated with mitochondrial diseases like Leigh syndrome and other dysfunctions involving oxidative phosphorylation. Complex I is a crucial metabolic enzyme and a validated drug target in contexts like neurodegeneration and metabolic diseases.

Other names

Complex I iron-sulfur proteinNADH:ubiquinone oxidoreductase iron-sulfur proteinNDUFS (plus specific numbers: NDUFS1, NDUFS4, etc.)CI-13kD-A (for NDUFS6)CI-18 (for NDUFS4)CI-23k (for NDUFS8)TYKY (NDUFS8)

Inhibition of Complex I (blocks electron transfer, reduces ATP generation, can trigger cell death); Modulation of redox/ROS balance (by altering electron flow)