Article
Ptch2 loss drives myeloproliferation and myeloproliferative neoplasm progression.
The Journal of experimental medicine - 8 Feb 2016
Klein Claudius, Zwick Anabel, Kissel Sandra, Forster Christine Ulrike, Pfeifer Dietmar, Follo Marie, Illert Anna Lena, Decker Sarah, Benkler Thomas, Pahl Heike, Oostendorp Robert A J, Aumann Konrad, Duyster Justus, Dierks Christine
Abstract excerpt
JAK2V617F(+) myeloproliferative neoplasms (MPNs) frequently progress into leukemias, but the factors driving this process are not understood. Here, we find excess Hedgehog (HH) ligand secretion and loss of PTCH2 in myeloproliferative disease, which drives canonical and noncanonical HH-signaling. Interestingly, Ptch2(-/-) mice mimic dual pathway activation and develop a MPN-phenotype with leukocytosis (neutrophils...
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