IDH1 and IDH2 have critical roles in 2-hydroxyglutarate production in D-2-hydroxyglutarate dehydrogenase depleted cells

► D2HGDH is a causative gene of D-2-hydroxyglutaric aciduria type I. ► Knockdown of D2HGDH increased 2-hydroxyglutarate in cells. ► Knockdown of IDH1 and IDH2 decreased 2-hydroxyglutarate in D2HGDH depleted cells. ► Expression of IDH1 or IDH2 increased 2-hydroxyglutarate in cells.