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FOP-FGFR1 tyrosine kinase, the product of a t(6;8) translocation, induces a fatal myeloproliferative disease in mice.

Authors: Guasch, G  Delaval, B  Arnoulet, C  Xie, MJ  Xerri, L  Sainty, D  Birnbaum, D  Pebusque, MJ 
Citation: Guasch G, etal., Blood. 2004 Jan 1;103(1):309-12. Epub 2003 Sep 11.
Pubmed: (View Article at PubMed) PMID:12969958
DOI: Full-text: DOI:10.1182/blood-2003-05-1690

Constitutive activation of aberrant fibroblast growth factor receptor 1 (FGFR1) kinase as a consequence of gene fusion such as FOP-FGFR1 associated with t(6; 8)(q27;p11-12) translocation, is the hallmark of an atypical aggressive stem cell myeloproliferative disorder (MPD) in humans. In this study, we show that expression of FOP-FGFR1 in primary bone marrow cells induced by retroviral transduction generates a MPD in mice. Constitutive FOP-FGFR1 kinase activity was both essential and sufficient to cause a chronic myeloproliferative syndrome in the murine bone marrow transplantation model. In contrast to the human disorder, lymphoproliferation and progression to acute phase were not observed. Lymphoid symptoms, however, appeared when onset of the disease was delayed as the result of mutation of FOP-FGFR1 at tyrosine 511, the phospholipase C gamma (PLCgamma) binding site.

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RGD ID: 11352667
Created: 2016-07-14
Species: All species
Last Modified: 2016-07-14
Status: ACTIVE



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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.