IDH1 (isocitrate dehydrogenase 1) catalyzes the NADP(+)-dependent oxidative decarboxylation of isocitrate to 2-ketoglutarate, generating NADPH—a critical cofactor for biosynthetic pathways 1. The enzyme functions in cytosolic and mitochondrial compartments, participating in the tricarboxylic acid cycle and cellular metabolism. Wild-type IDH1 is essential for normal metabolic homeostasis. However, somatic IDH1 mutations—particularly R132H—occur frequently in gliomas (77% of diffuse astrocytomas, 89% of oligodendrogliomas) 2. These mutations cause a neomorphic enzyme activity that converts α-ketoglutarate to 2-hydroxyglutarate (2HG), an oncometabolite driving tumorigenesis 3. IDH1 mutations are similarly common in cytogenetically normal acute myeloid leukemia and associated cartilaginous tumors, characterizing Ollier disease and Maffucci syndrome 4. Clinically, IDH1 mutation status serves as a prognostic biomarker: glioma patients with IDH1 mutations show significantly improved overall and progression-free survival 2. Accordingly, IDH1 inhibitors like olutasidenib have been FDA-approved for treating relapsed/refractory acute myeloid leukemia with susceptible IDH1 mutations 5. Conversely, the IDH1-R132H mutation aggravates cisplatin-induced kidney injury through ferroptosis mechanisms 6, highlighting context-dependent clinical significance.