IDH2 (Isocitrate Dehydrogenase 2) is an enzyme that plays a crucial role in cellular metabolism. It is a key enzyme in the citric acid cycle (also known as the Krebs cycle or TCA cycle), catalyzing the oxidative decarboxylation of isocitrate to α-ketoglutarate (α-KG) while reducing NADP+ to NADPH. This process is essential for cellular energy metabolism. Mutations in the IDH2 are associated with the development and progression of various types of cancer. These mutations confer a new enzymatic activity on IDH2, converting α-KG to 2-hydroxyglutarate (2-HG), an oncometabolite that accumulates and disrupts epigenetic regulation and gene expression, promoting tumorigenesis.