| Summary: | <p>The gene mutations of isocitrate dehydrogenase 1 and 2 (<em>IDH1/2</em>) mainly occur in astrocytoma, anaplastic astrocytoma, oligodendroglioma, anaplastic oligodendroglioma, oligoastrocytoma, anaplastic oligoastrocytoma and secondary glioblastoma. The <em>IDH1/2</em> gene mutation can alter proteinase function, consume α-ketoglutarate and nicotinamide adenine dinucleotide phosphate-reduced (NADPH) and thus produce carcinogenic metabolite, 2-hydroxyglutarate. The intracellular accumulation of 2-hydroxyglutarate will induce a series of downstream effects which may result in the development of gliomas mentioned above. Both <em>IDH1/2</em> mutations and other concomitant hereditary variations are biomarkers for differential diagnosis and <em>IDH1/2</em> mutations are also independent factors for the prognosis of gliomas. The molecular targeting therapy for <em>IDH1/2</em> mutations has become the research focus of glioma treatment. This review summarizes the recent progress of this field.</p><p> </p><p><strong>DOI: </strong>10.3969/j.issn.1672-6731.2015.11.017</p>
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