The primary mechanism by which mutant IDH contributes to the pathogenesis of GBM is ascribed to the deregulated enzymatic activity of mutant IDH, which converts αKG into the metabolite D2HG, which in turn inhibits αKG-dependent dioxygenases, such as ten–eleven translocation (TET) family 5-methylcytosine DNA hydroxylases and the Jumonji C domain-containing histone–lysine demethylases (KDMs) (Xiao et al., 2012; Miller et al., 2017). Here, IDH1 is linked to glioblastoma.