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. 2014 Oct 13;6(11):1359–1370. doi: 10.15252/emmm.201302627

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© 2014 The Authors. Published under the terms of the CC BY 4.0 license

This is an open access article under the terms of the Creative Commons Attribution 4.0 License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

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Wild-type IDH-1 converts isocitrate to alpha-ketoglutarate. Heterozygotic IDH-1 (R132H) mutant protein can convert alpha-ketoglutarate to 2-hydroxyglutarate, an onco-metabolite. Selective therapeutics against IDH-1 (R132H) prevent 2-hydroxyglutarate production while leaving normal IDH-1 enzymatic function intact.