Mutations in enzymes regulating metabolite flux are implicated in gliomas development, as highlighted by the discovery of isocitrate dehydrogenase 1 (IDH1), in more than 70% of diffusely infiltrating WHO grade II and grade III astrocytic and oligodendroglial gliomas, as well as in a small fraction of glioblastomas (GBM), particularly those that develop from low-grade-gliomas (LGG) [3,5,6,7,8]. The gene discussed is IDH1; the disease is glioma.