Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial (IDH3A)

Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial (IDH3A), is the catalytic alpha subunit of the NAD+-dependent isocitrate dehydrogenase heterotetramer in mitochondria. This enzyme catalyzes the irreversible oxidative decarboxylation of isocitrate to alpha-ketoglutarate in the tricarboxylic acid (TCA) cycle, generating NADH for ATP production. IDH3A is essential for cellular energy metabolism. It is regulated allosterically by cellular energy status: activated by ADP and inhibited by ATP/NADH. Mutations in IDH3A cause severe inherited diseases such as autosomal recessive retinitis pigmentosa and severe infantile encephalopathy, as well as being implicated in cancer, psychiatric, and metabolic disorders. IDH3A is considered a putative cancer therapeutic target because its overexpression is associated with poor prognosis in certain tumors. Pharmacological inhibition of IDH3A is not established and would carry risks of severe mitochondrial dysfunction[1][2][3][4].

Other names

Isocitrate dehydrogenase 3 catalytic subunit alphaIsocitric dehydrogenase subunit alphaNAD(H)-specific isocitrate dehydrogenase alpha subunitIsocitrate dehydrogenase (NAD+) alpha chainIsocitrate dehydrogenase (NAD(+)) 3 alphaIsocitrate dehydrogenase 3 (NAD+) alphaNAD+-specific ICDH alpha subunitH-IDH alphaRP90Isocitrate dehydrogenase (NAD(+)) 3 catalytic subunit alpha

For potential agents: Inhibition of catalytic activity, leading to reduced α-ketoglutarate synthesis and cellular energy perturbation (hypothetical; not therapeutically validated[1][3]).