The observations that the IDH2R140Q inhibitor, AGI-026, rescued cardiomyopathy and provided a survival benefit in this model through 2HG reduction, and potently inhibited 2HG production in lymphoblasts from D2HGA patients carrying IDH2R140Q mutations, suggest that mutant IDH2 inhibition has therapeutic potential in D2HGA type II. Here, IDH2 is linked to cardiomyopathy.