Isocitrate dehydrogenase (IDH) is a group of key metabolic enzymes involved in the tricarboxylic acid cycle (TCA cycle), catalyzing the conversion of isocitrate to α-ketoglutarate. The IDH family includes IDH1, IDH2, and IDH3. IDH1 and IDH2 are found in the cytoplasm and mitochondria, primarily participating in the production of NADPH, while IDH3 operates in the mitochondria, contributing to energy production. IDH enzymes are expressed in a wide range of tissues and are closely related to energy metabolism, redox balance, and cell signaling. Mutations in specific IDH genes, especially those in IDH1 and IDH2, are associated with the development of various cancers. These mutations can lead to the abnormal accumulation of the metabolic product 2-hydroxyglutarate (2-HG), affecting cell differentiation and proliferation.