Physiological Jak2V617F Expression Causes a Lethal Myeloproliferative Neoplasm with Differential Effects on Hematopoietic Stem and Progenitor Cells

Ann Mullally, Steven W. Lane, Brian Ball, Christine Megerdichian, Rachel Okabe, Fatima Al-Shahrour, Mahnaz Paktinat, J. Erika Haydu, Elizabeth Housman, Allegra M. Lord, Gerlinde Wernig, Michael G. Kharas, Thomas Mercher, Jeffery L. Kutok, D. Gary Gilliland, Benjamin L. Ebert

    Résultats de recherche: Contribution à un journalArticleRevue par des pairs

    315 Citations (Scopus)

    Résumé

    We report a Jak2V617F knockin mouse myeloproliferative neoplasm (MPN) model resembling human polycythemia vera (PV). The MPN is serially transplantable and we demonstrate that the hematopoietic stem cell (HSC) compartment has the unique capacity for disease initiation but does not have a significant selective competitive advantage over wild-type HSCs. In contrast, myeloid progenitor populations are expanded and skewed toward the erythroid lineage, but cannot transplant the disease. Treatment with a JAK2 kinase inhibitor ameliorated the MPN phenotype, but did not eliminate the disease-initiating population. These findings provide insights into the consequences of JAK2 activation on HSC differentiation and function and have the potential to inform therapeutic approaches to JAK2V617F-positive MPN.

    langue originaleAnglais
    Pages (de - à)584-596
    Nombre de pages13
    journalCancer Cell
    Volume17
    Numéro de publication6
    Les DOIs
    étatPublié - 15 juin 2010

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