While there is overwhelming evidence that D-2HG acts as an oncometabolite in cells that express mutant IDH1/IDH2, there is limited evidence indicating that changes in D2hgdh expression or activity drive glioma progression, with only a handful of studies hinting this possibility (Lin et al. 2015; Eckel-Passow et al. 2020). This evidence concerns the gene IDH2 and central nervous system cancer.