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IDH-305 is an orally available, mutation-selective, and brain-penetrant IDH1 inhibitor targeting the IDH1 (R132) mutation. IDH-305 is 200-fold more selective for mutant IDH1 isoforms than wild type with IC50s of 27 nM,28 nM and 6.14 nM for IDH1R132H, IDH1R132C and IDH1WT, respectively.