RNA splicing as a therapeutic target in myelodysplastic syndromes

Semin Hematol. 2024 Dec;61(6):431-441. doi: 10.1053/j.seminhematol.2024.10.005. Epub 2024 Oct 23.

Abstract

Myelodysplastic syndromes (MDS) represent a heterogeneous group of hematological disorders and are more commonly found in people over the age of 60. MDS patients exhibit peripheral blood cytopenias and carry an increased risk of disease progression to acute myeloid leukemia (AML). Splicing factor mutations (including genes SF3B1, SRSF2, U2AF1, and ZRSR2) are early events identified in more than 50% of MDS cases. These mutations cause aberrant pre-mRNA splicing and impact MDS pathophysiology. Emerging evidence shows that splicing factor-mutant cells are more sensitive to perturbations targeting the spliceosome, aberrantly spliced genes and/or their regulated molecular pathways. This review summarizes current therapeutic strategies and ongoing efforts targeting splicing factor mutations for the treatment of MDS.

Keywords: Clinical trial; Myelodysplastic syndromes; Spliceosome; Splicing factor.

Publication types

  • Review

MeSH terms

  • Humans
  • Molecular Targeted Therapy / methods
  • Mutation*
  • Myelodysplastic Syndromes* / drug therapy
  • Myelodysplastic Syndromes* / genetics
  • Myelodysplastic Syndromes* / metabolism
  • RNA Splicing Factors / genetics
  • RNA Splicing Factors / metabolism
  • RNA Splicing* / genetics
  • Spliceosomes / genetics
  • Spliceosomes / metabolism

Substances

  • RNA Splicing Factors