Biology of IDH mutant cholangiocarcinoma

• Published in: Hepatology (Baltimore, Md.) Vol. 75; no. 5; pp. 1322 - 1337
• Main Authors: Wu, Meng‐Ju, Shi, Lei, Merritt, Joshua, Zhu, Andrew X., Bardeesy, Nabeel
• Format: Journal Article
• Language: English
• Published: United States Wolters Kluwer Health, Inc 01.05.2022
• Subjects: Bile Duct Neoplasms - genetics; Bile Ducts, Intrahepatic - metabolism; Biology; Cell differentiation; Cholangiocarcinoma; Cholangiocarcinoma - genetics; DNA repair; Enzymes; Epigenetics; Hepatology; Humans; Isocitrate dehydrogenase; Isocitrate Dehydrogenase - genetics; Mutants; Mutation; Therapeutic targets; Tumors

Isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) are the most frequently mutated metabolic genes across human cancers. These hotspot gain‐of‐function mutations cause the IDH enzyme to aberrantly generate high levels of the oncometabolite, R‐2‐hydroxyglutarate, which competitively inhibits enzymes th...