Mitochondrial NADH Ubiquinone Oxidoreductase Complex I (Complex I)

Mitochondrial NADH ubiquinone oxidoreductase, commonly known as Complex I, is the first and largest enzyme complex of the mitochondrial respiratory chain. It plays a central role in cellular energy metabolism by catalyzing electron transfer from NADH to ubiquinone (coenzyme Q10), coupled with proton translocation across the inner mitochondrial membrane. Defects or mutations in complex I subunits cause various inherited neuromuscular and metabolic disorders such as Leigh syndrome, Parkinson’s disease, ischemia/reperfusion injury after stroke or heart attack, among others. Dysfunctional complex I is also implicated in aging-related degenerative diseases due to increased ROS production.

Other names

Respiratory complex IType I NADH dehydrogenaseNDHI

Inhibition of electron transfer from NADH to ubiquinone