NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 (NDUFA13)

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 (NDUFA13) is an accessory subunit of mitochondrial Complex I, the largest enzyme of the electron transport chain responsible for ATP generation by oxidative phosphorylation[1][2][3]. While not directly involved in catalysis, NDUFA13 is critical for the proper assembly and stability of Complex I, ensures electron transfer from NADH to ubiquinone, and supports mitochondrial membrane potential[1][3]. Also known as GRIM-19, it is implicated in cell death regulation, immune defense (particularly mucosal response to bacteria), and inhibition of STAT3-mediated transcription[1][2][3]. Its altered expression is linked to diseases including inflammatory bowel disease and possibly disorders involving mitochondrial dysfunction or apoptosis[1]. Flurpiridaz F-18 is an approved inhibitor that targets this protein, while NADH interacts physiologically as a substrate[3].

Other names

Cell death regulatory protein GRIM-19GRIM-19GRIM19CI-B16.6Complex I-B16.6Gene associated with retinoic and IFN-induced mortality 19 proteinNADH-ubiquinone oxidoreductase B16.6 subunit

Inhibition of electron transfer (by Flurpiridaz F-18) and modulation of Complex I activity affecting ATP production and cell viability.