Kats et al. developed a mouse transgenic model of IDH2-R140Q mutation that has the capacity to be both tissue-specific and on/off inducible; using this genetic model, it was demonstrated that expression of the transgene elicited an on/off inducible 2-HG production that was comparable to that observed in AML patients [104]. The gene discussed is IDH2; the disease is acute myeloid leukemia.