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IDH mutations and production of D-2-HG. IDH1 and IDH2 are NADP+ dependent enzymes and distribute in the cytosol and mitochondria, respectively. IDH3 is a NAD+ dependent enzyme that locates in mitochondria. Mutations of IDH1 and IDH2 enzymes are sufficient to convert a-KG to D-2HG. NADP, nicotinamide adenine dinucleotide phosphate, NADPH, the reduced form of NADP.
