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| A JAK2 mutation in myeloproliferative disorders: pathogenesis and therapeutic and scientific prospects |
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| Author(s): James C, Ugo V, Casadevall N, Constantinescu SN, Vainchenker W |
| Source: TRENDS IN MOLECULAR MEDICINE Volume: 11 Issue: 12 Pages: 546-554 Published: DEC 2005 |
| Times Cited: 39 References: 69 |
| Abstract: Myeloproliferative disorders include several pathologies sharing the common feature of being clonal hematopoietic stem cell diseases. The molecular basis of chronic myeloid leukemia was characterized many years ago with the discovery of the t(9;22) translocation and its product the BCR-ABL oncoprotein. The recent finding of a recurrent mutation in the Janus 2 tyrosine kinase gene is a major advance in our understanding of the pathogenesis of several other myeloproliferative disorders, including polycythemia vera, essential thrombocythemia and idiopathic myelofibrosis. Although this work clearly identifies a frequent (similar to 50%) subgroup of myeloproliferative disorders and explains most biological abnormalities described so far, it also raises the major question of how a single mutation can explain disease heterogeneity. Such a recurrent and unique mutation leading to a tyrosine kinase deregulation would make a suitable target for the development of specific therapies. |
| Document Type: Review |
| Language: English |
| Reprint Address: James, C (reprint author), Univ Paris 11, Inst Gustave Roussy, INSERM, U362, 39 Rue Camille Desmoulins, F-94805 Villejuif, France |
Addresses:
1. Univ Paris 11, Inst Gustave Roussy, INSERM, U362, F-94805 Villejuif, France
2. CHU Brest, Lab Hematol, F-29609 Brest, France
3. Hop Hotel Dieu, Lab Hematol, F-75181 Paris, France
4. Univ Catholique Louvain, Ludwig Inst Canc Res, B-1200 Brussels, Belgium
5. Univ Catholique Louvain, Christian Duve Inst Cellular Pathol, B-1200 Brussels, Belgium
6. Univ Catholique Louvain, MEXP Unit, B-1200 Brussels, Belgium |
| Publisher: ELSEVIER SCI LTD, THE BOULEVARD, LANGFORD LANE, KIDLINGTON, OXFORD OX5 1GB, OXON, ENGLAND |
| Subject Category: Biochemistry & Molecular Biology; Cell Biology; Medicine, Research & Experimental |
| IDS Number: 998LS |
| ISSN: 1471-4914 |
| DOI: 10.1016/j.molmed.2005.10.003 |
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