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Site-specific methylation of SRSF2 P95H by SETD2 inhibits MDSC-mediated proinflammatory niche formation in mouse models of myelodysplastic syndrome
Editor’s summary Myelodysplastic syndrome (MDS) is a clonal hematopoietic malignancy and has a high risk of progression to acute myeloid leukemia (AML). Here, Li et al. evaluate the underlying mechanism by which patients with SRSF2 mutations are at greater risk of MDS to AML transformation. They identify that SETD2 epigenetically inhibits mutant SRSF2 pathogenic splicing that induces an inflammatory bone marrow microenvironment that promotes MDS development.
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