Mutations at the Arg132 position inthe active site impart theability to convert αKG to 2-hydroxyglutarate (2-HG),85 an oncometabolite that promotes stemness inhuman cells and inhibits DNA demethylases.86,87 Such variants are involved in the progress of low-grade glioma,glioblastoma, and acute myeloid leukemia (AML).88 Describing wild-type and variant IDH1 reaction mechanismsmay help design transition-state analogs that act as selective inhibitorsof mutant IDH1 and are able to interfere with such diseases. This evidence concerns the gene IDH1 and glioblastoma.