NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 (NDUFA1)

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 (NDUFA1) is a small accessory subunit of mitochondrial complex I, located within the inner mitochondrial membrane. While not directly involved in catalysis, it facilitates complex assembly, stabilizes structural organization, helps anchor complex I, and contributes indirectly to proton translocation during electron transfer from NADH to ubiquinone. Dysfunction or mutation of NDUFA1 can impair ATP production, increase ROS, and is causative in rare mitochondrial disorders such as complex I deficiency and Leigh syndrome. Complex I activity and associated ROS generation are of clinical importance; numerous drugs and toxins (e.g., metformin, rotenone, paraquat) interact with complex I—sometimes targeting NDUFA1—and carry therapeutic or safety implications.

Other names

CI-MWFEComplex I-MWFENADH-ubiquinone oxidoreductase MWFE subunitMWFEMC1DN12Complex I MWFE subunitZNF183NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1NADH:ubiquinone oxidoreductase MWFE subunit

Inhibitors (Metformin, Rotenone): reduce electron flow through complex I, decrease ATP synthesis, increase mitochondrial ROS generation, trigger apoptosis in high doses/contexts