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. 2020 Nov 21;11(11):998. doi: 10.1038/s41419-020-03196-0

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© The Author(s) 2020

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

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Fig. 1 — IDH wild-type converts isocytrate into a-KG, whereas IDH1^Mut changes the enzymatic capacity to further convert a-KG into 2-HG, that accumulates inside the cell. Various cellular and molecular effects of D2HG accumulation have been identified amongst them the most prominent are depictured. Those changes open opportunities for IDH1^Mut specific therapeutic interventions and new diagnostic modalities.