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Blood, 1 June 2004, Vol. 103, No. 11, pp. 4243-4250. Prepublished online as a Blood First Edition Paper on February 24, 2004; DOI 10.1182/blood-2003-08-2650. This Article Full Text (PDF) All Versions of this Article: 2003-08-2650v1 103/11/4243    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Le, D. T Articles by Shannon, K. M Search for Related Content PubMed PubMed Citation Articles by Le, D. T Articles by Shannon, K. M Social Bookmarking                   What's this? Submitted August 7, 2003 Accepted December 4, 2003 Somatic Inactivation of Nf1 in Hematopoietic Cells Results in a Progressive Myeloproliferative Disorder Doan T Le, Namie Kong, Yuan Zhu, Jennifer O Lauchle, Abigail Aiyigari, Benjamin S Braun, Endi Wang, Scott C Kogan, Michelle M Le Beau, Luis Parada, and Kevin M Shannon* Pediatrics, University of California, San Francisco, San Francisco, CA, USA University of Texas Southwestern, Dallas, TX, USA Laboratory Medicine, University of California, San Francisco, San Francisco, CA, USA Medicine, University of Chicago, Chicago, IL, USA * Corresponding author; email: kevins{at}itsa.ucsf.edu. The NF1 tumor suppressor gene encodes a GTPase activating protein that negatively regulates Ras signaling and is inactivated in a subset of juvenile myelomonocytic leukemias (JMMLs). Adoptive transfer of fetal liver cells from Nf1 mutant mice models JMML; however, this system has important limitations as a platform for performing biologic and preclinical studies. We have exploited the interferon-inducible Mx1-Cre transgene to ablate a conditional mutant Nf1 allele in hematopoietic cells. Somatic inactivation of Nf1 induces a myeloproliferative disorder with 100% penetrance that is associated with a subacute clinical course, tissue infiltration by myeloid cells, hypersensitivity to granulocyte-macrophage colony stimulating factor, hyperproliferation, and resistance to apoptosis. These Mx1-Cre, Nf1flox/flox mice establishes a tractable experimental model for testing therapeutics and for identifying mutations that cooperate with hyperactive Ras in myeloid leukemogenesis. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: E. A. Lasater, W. K. Bessler, L. E. Mead, W. E. Horn, D. W. 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