Beyond DDX41, mutations in genes that encode RNA splicing-related factors implicated in myeloid malignancies are observed in about 40–60% of MDS patients (52, 53); of note, frequently mutated genes (namely, SF3B1, SRSF2 and U2AF1) all encode factors involved in the recognition of the 3’SS (Figure 2B) (54). Here, DDX41 is linked to myelodysplastic syndrome.