In fact, the expression of mutant IDH1 in progenitors of the subventricular zone induces a pretumorigenic state [109]; mutant IDH1 in cooperation with mutant TP53 and ATRX more efficiently promoted gliomagenesis, blocking neural stem cell differentiation via repression of SOX2 and induced a transcriptional profile resembling IDH-mutant human low-grade gliomas [110]. Here, SOX2 is linked to central nervous system cancer.