摘要
Cytosolic isocitrate dehydrogenase 1(IDH1)and its mitochondrial counterpart,IDH2,are critical TCA cycle enzymes that catalyze the oxidative decarboxylation of isocitrate to produce alpha-ketoglutarate(a-KG).Mutations in IDH1/2 occur in∼80%of grade II-III gliomas and secondary glioblastomas,^(1–3) and in 10%to 20%of acute myeloid leukemia(AML).^(4–6) To date,all identified mutations in IDH1/2 are heterozygous,missense mutations,leading to the substitution of arginine 132 in IDH1 and arginine 172 or 140 in IDH2.
