Journal article
Pathogenesis of myeloproliferative neoplasms.
- Skoda RC Department of Biomedicine, Experimental Hematology, University Hospital Basel, Basel, Switzerland. Electronic address: radek.skoda@unibas.ch.
- Duek A Hematology Institute, Chaim Sheba Medical Center, Tel Hashomer, Ramat Gan, Israel.
- Grisouard J Department of Biomedicine, Experimental Hematology, University Hospital Basel, Basel, Switzerland.
- 2015-07-26
Published in:
- Experimental hematology. - 2015
Animals
Gene Expression Regulation, Neoplastic
Hematologic Neoplasms
Humans
Mutation
Myeloproliferative Disorders
Neoplasm Proteins
Signal Transduction
English
Major progress has been recently made in understanding the molecular pathogenesis of myeloproliferative neoplasms (MPN). Mutations in one of four genes-JAK2, MPL, CALR, and CSF3R-can be found in the vast majority of patients with MPN and represent driver mutations that can induce the MPN phenotype. Hyperactive JAK/STAT signaling appears to be the common denominator of MPN, even in patients with CALR mutations and the so-called "triple-negative" MPN, where the driver gene mutation is still unknown. Mutations in epigenetic regulators, transcription factors, and signaling components modify the course of the disease and can contribute to disease initiation and/or progression. The central role of JAK2 in MPN allowed development of small molecular inhibitors that are in clinical use and are active in almost all patients with MPN. Advances in understanding the mechanism of JAK2 activation open new perspectives of developing the next generation of inhibitors that will be selective for the mutated forms of JAK2.
- Language
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- English
- Open access status
- hybrid
- Identifiers
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- DOI 10.1016/j.exphem.2015.06.007
- PMID 26209551
- Persistent URL
- https://sonar.ch/global/documents/102052
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