Certain gliomas have somatic mutation in cytosolic isocitrate dehydrogenase 1. Dang et al. have shown that these mutations confer the enzyme the new ability to transform alpha-ketoglutarate in 2-hydroxyglutarate. This metabolite might be oncogenic, although this is not proven yet.
Dang L, White DW, Gross S, et al. Cancer-associated IDH1 mutations produce 2-hydroxyglutarate. Nature 2009;462:739-744