IDH305 is another orally bioavailable IDH1 inhibitor with selective inhibition against IDH1-R132H mutant and has shown efficacy in preclinical studies.205 In a phase I trial of patients with advanced malignancies with IDH1 R132H mutations (glioma, AML, MDS, non-CNS solid tumors, and unknown), IDH305 was administered at varying doses twice daily. Here, IDH1 is linked to myelodysplastic syndrome.