A small molecule inhibitor of mutant IDH2 rescues cardiomyopathy in a D-2-hydroxyglutaric aciduria type II mouse model.

doi:10.1007/s10545-016-9960-y

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Article

A small molecule inhibitor of mutant IDH2 rescues cardiomyopathy in a D-2-hydroxyglutaric aciduria type II mouse model.

Journal of inherited metabolic disease - 1 Nov 2016

Wang Fang, Travins Jeremy, Lin Zhizhong, Si Yaguang, Chen Yue, Powe Josh, Murray Stuart, Zhu Dongwei, Artin Erin, Gross Stefan, Santiago Stephanie, Steadman Mya, Kernytsky Andrew, Straley Kimberly, Lu Chenming, Pop Ana, Struys Eduard A, Jansen Erwin E W, Salomons Gajja S, David Muriel D, Quivoron Cyril, Penard-Lacronique Virginie, Regan Karen S, Liu Wei, Dang Lenny, Yang Hua, Silverman Lee, Agresta Samuel, Dorsch Marion, Biller Scott, Yen Katharine, Cang Yong, Su Shin-San Michael, Jin Shengfang

Abstract excerpt

D-2-hydroxyglutaric aciduria (D2HGA) type II is a rare neurometabolic disorder caused by germline gain-of-function mutations in isocitrate dehydrogenase 2 (IDH2), resulting in accumulation of D-2-hydroxyglutarate (D2HG). Patients exhibit a wide spectrum of symptoms including cardiomyopathy, epilepsy, developmental delay and limited life span. Currently, there are no effective therapeutic interventions. We...

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PMID: 27469509
DOI: 10.1007/s10545-016-9960-y
PMCID: PMC5065612

Genes

IDH2

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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