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NOTCH signaling in the pathogenesis of splenic marginal zone lymphoma-opportunities for therapy
- Source :
- Leukemialymphoma. 63(2)
- Publication Year :
- 2021
-
Abstract
- NOTCH signaling is a highly conserved pathway mediated by four receptors (NOTCH 1-4) playing critical functions in proliferation, differentiation, and cell death. Under physiologic circumstances, NOTCH2 is a key regulator in marginal zone differentiation and development. Over the last decade, growing data demonstrated frequent NOTCH2 mutations in splenic marginal zone lymphoma (SMZL) underscoring its critical role in the pathogenesis of this disease. Moreover, NOTCH2 specificity across studies supports the rationale to assess its value as a diagnosis biomarker in a disease without pathognomonic features. These data make NOTCH signaling an appealing target for drug discovery in SMZL; however, prior efforts attempting to manipulate this pathway failed to demonstrate meaningful clinical benefit, or their safety profile prevented further development. In this review, we discuss the current knowledge of NOTCH implications in the pathogenesis and as a potential druggable target in SMZL.
- Subjects :
- Cancer Research
Lymphoma
Splenic Neoplasms
Druggability
Regulator
Notch signaling pathway
Hematology
Disease
Lymphoma, B-Cell, Marginal Zone
Biology
medicine.disease
Marginal zone
Leukemia, Lymphocytic, Chronic, B-Cell
Pathogenesis
Oncology
Mutation
Cancer research
medicine
Biomarker (medicine)
Humans
Splenic marginal zone lymphoma
Signal Transduction
Subjects
Details
- ISSN :
- 10292403
- Volume :
- 63
- Issue :
- 2
- Database :
- OpenAIRE
- Journal :
- Leukemialymphoma
- Accession number :
- edsair.doi.dedup.....eba47ac784c7c58b4ee90f0389a22f51