Article
Gray platelet syndrome: proinflammatory megakaryocytes and α-granule loss cause myelofibrosis and confer metastasis resistance in mice.
Blood - 4 Dec 2014
Guerrero Jose A, Bennett Cavan, van der Weyden Louise, McKinney Harriet, Chin Melody, Nurden Paquita, McIntyre Zoe, Cambridge Emma L, Estabel Jeanne, Wardle-Jones Hannah, Speak Anneliese O, Erber Wendy N, Rendon Augusto, Ouwehand Willem H, Ghevaert Cedric
Abstract excerpt
NBEAL2 encodes a multidomain scaffolding protein with a putative role in granule ontogeny in human platelets. Mutations in NBEAL2 underlie gray platelet syndrome (GPS), a rare inherited bleeding disorder characterized by a lack of α-granules within blood platelets and progressive bone marrow fibrosis. We present here a novel Nbeal2(-/-) murine model of GPS and demonstrate that the lack of α-granules is due to...
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