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http://purl.uniprot.org/citations/25258341http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/25258341http://www.w3.org/2000/01/rdf-schema#comment"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 their loss from platelets/mature megakaryocytes (MKs), and not by initial impaired formation. We show that the lack of Nbeal2 confers a proinflammatory phenotype to the bone marrow MKs, which in combination with the loss of proteins from α-granules drives the development of bone marrow fibrosis. In addition, we demonstrate that α-granule deficiency impairs platelet function beyond their purely hemostatic role and that Nbeal2 deficiency has a protective effect against cancer metastasis."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.org/dc/terms/identifier"doi:10.1182/blood-2014-04-566760"xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/author"Speak A.O."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/author"Rendon A."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/author"Chin M."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/author"van der Weyden L."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/author"Bennett C."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/author"Ouwehand W.H."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/author"Erber W.N."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/author"Nurden P."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/author"Ghevaert C."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/author"Guerrero J.A."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/author"Wardle-Jones H."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/author"Estabel J."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/author"McKinney H."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/author"Cambridge E.L."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/author"McIntyre Z."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/date"2014"xsd:gYear
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/name"Blood"xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/pages"3624-3635"xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/title"Gray platelet syndrome: proinflammatory megakaryocytes and alpha-granule loss cause myelofibrosis and confer metastasis resistance in mice."xsd:string
http://purl.uniprot.org/citations/25258341http://purl.uniprot.org/core/volume"124"xsd:string
http://purl.uniprot.org/citations/25258341http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/25258341
http://purl.uniprot.org/citations/25258341http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/25258341