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MPLW515L Is a Novel Somatic Activating Mutation in Myelofibrosis with Myeloid Metaplasia

PLoS Medicine · 2006 · Vol. 3(7) · pp. e270–e270

Yana Pikman(Brigham and Women's Hospital)Benjamin H. Lee(Harvard University)Thomas Mercher(Brigham and Women's Hospital)Elizabeth McDowell(Harvard University)Benjamin L. Ebert(Broad Institute)Maricel Gozo(Brigham and Women's Hospital)Adam Cuker(Brigham and Women's Hospital)Gerlinde Wernig(Harvard University)Sandra Moore(Harvard University)Ilene Galinsky(Dana-Farber Cancer Institute)Daniel J. DeAngelo(Harvard University)Jennifer Clark(Dana-Farber Cancer Institute)Stephanie J. Lee(Harvard University)Todd R. Golub(Dana-Farber Cancer Institute)Martha Wadleigh(Harvard University)D. Gary Gilliland(Harvard University)Ross L. Levine✉(Dana-Farber Cancer Institute)

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Abstract

Activation of JAK-STAT signaling via MPLW515L is an important pathogenetic event in patients with JAK2V617F-negative MF. The bone marrow transplant model of MPLW515L-mediated myeloproliferative disorders (MPD) exhibits certain features of human MF, including extramedullary hematopoiesis, splenomegaly, and megakaryocytic proliferation. Further analysis of positive and negative regulators of the JAK-STAT pathway is warranted in JAK2V617F-negative MPD.

Myeloproliferative Neoplasms: Diagnosis and Treatment Eosinophilic Disorders and Syndromes Kruppel-like factors researchThrombopoietin receptorErythropoietin receptorBiologyCancer researchMyelofibrosisThrombopoietinMyeloidHaematopoiesisSignal transductionBone marrow

MeSH terms

AnimalsBone Marrow CellsCell DivisionCells, CulturedColony-Forming Units AssayDisease Models, AnimalGene Expression RegulationGenetic VectorsHematopoiesisHumansMegakaryocytesMice, Inbred BALB CMyeloproliferative DisordersPhosphorylationProtein Processing, Post-Translational