[3] Isocitrate dehydrogenase (IDH) enzyme catalyzes the oxidative decarboxylation of isocitrate to produce α-ketoglutartate and at the same time use NADP+ as a cofactor to generate NADPH and maintain cellular redox status.[4] IDH1 mutations occurred in vast majority of World Health Organization (WHO) grade II/III gliomas and secondary glioblastomas. The gene discussed is IDH3A; the disease is central nervous system cancer.