NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial (NDUFS1)

NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial (NDUFS1), also known as NADH:ubiquinone oxidoreductase core subunit S1, is an iron-sulfur enzyme and the largest nuclear-encoded core subunit of mitochondrial complex I. Located in the inner mitochondrial membrane, this enzyme subunit plays a central role in the electron transport chain by transferring electrons from NADH to ubiquinone, facilitating ATP generation via oxidative phosphorylation. NDUFS1 is essential for normal mitochondrial function, energy metabolism, and regulation of reactive oxygen species. Disruption or mutation of NDUFS1 leads to mitochondrial complex I deficiency, implicated in a spectrum of human diseases including heart failure, encephalopathies like Leigh syndrome, and some neurodegenerative diseases. Targeting NDUFS1 or modulating its expression is being explored as a therapeutic strategy in cardiac and metabolic diseases[1][2][4].

Other names

NADH:ubiquinone oxidoreductase core subunit S1Ndufs1Complex I subunit S1

Inhibitors or modulators of complex I reduce electron transfer from NADH to ubiquinone, decreasing ATP production and potentially increasing ROS. Agents that stabilize or enhance NDUFS1 function may preserve mitochondrial respiratory activity and decrease ROS-induced apoptosis.