The prevalence of IDH mutation is high in grade II-III and secondary glioblastomas (70–80%), the most frequently (> 90%) mutated IDH isoform is the cytosolic IDH1 R132H [7, 8], that has gained a neomorphic activity resulting in conversion of α-ketoglutarate (aKG) to the oncometabolite D-2-hydroxyglutarate (2-HG). Here, IDH1 is linked to glioblastoma.