IDH2 and central nervous system cancer: Point mutations at the arginine 132 (IDH1) and at arginine 140 or arginine 172 (IDH2) in the active site of IDH enzyme alter catalytic activity of the enzyme and results in increased production of 2-hydroxyglutarate (2-HG) which is potentially associated with increased cancer risk and glioma progression (Dang et al., 2009).