The Journal of Experimental Medicine
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Published online 13 December 2005 doi:10.1084/jem.20050807
Rockefeller University Press, 0022-1007 $8.00
JEM, Volume 202, Number 12, 1635-1641
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BRIEF DEFINITIVE REPORT

 Mastocytosis in mice expressing human Kit receptor with the activating Asp816Val mutation

Jacques P. Zappulla1, Patrice Dubreuil2, Sabine Desbois1, Sébastien Létard2, Nadine Ben Hamouda3, Marc Daëron4, Georges Delsol1, Michel Arock3, and Roland S. Liblau1,5

1 Institut National de la Santé et de la Recherche Médicale (INSERM) U563, Centre de Physiopathologie de Toulouse Purpan, Purpan Hospital, 31000 Toulouse, France
2 INSERM U599, Marseille Institute for Cancer, 13009 Marseille, France
3 Centre National de la Recherche Scientifique UMR 8147, Cellular and Molecular Hematology Laboratory, Pharmacy University, 75006 Paris, France
4 Cellular and Molecular Allergology Laboratory, Pasteur Institute, 75015 Paris, France
5 Immunology Laboratory, Rangueil University Hospital, 31000 Toulouse, France

CORRESPONDENCE Roland S. Liblau: rolandliblau{at}hotmail.com

Mastocytosis is a rare neoplastic disease characterized by a pathologic accumulation of tissue mast cells (MCs). Mastocytosis is often associated with a somatic point mutation in the Kit protooncogene leading to an Asp/Val substitution at position 816 in the kinase domain of this receptor. The contribution of this mutation to mastocytosis development remains unclear. In addition, the clinical heterogeneity presented by mastocytosis patients carrying the same mutation is unexplained. We report that a disease with striking similarities to human mastocytosis develops spontaneously in transgenic mice expressing the human Asp816Val mutant Kit protooncogene specifically in MCs. This disease is characterized by clinical signs ranging from a localized and indolent MC hyperplasia to an invasive MC tumor. In addition, bone marrow–derived MCs from transgenic animals can be maintained in culture for >24 mo and acquire growth factor independency for proliferation. These results demonstrate a causal link in vivo between the Asp816Val Kit mutation and MC neoplasia and suggest a basis for the clinical heterogeneity of human mastocytosis.


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