Abnormal proplatelet formation and emperipolesis in cultured human megakaryocytes from gray platelet syndrome patients
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منابع مشابه
Corrigendum: Abnormal proplatelet formation and emperipolesis in cultured human megakaryocytes from gray platelet syndrome patients
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متن کاملAbnormal proplatelet formation and emperipolesis in cultured human megakaryocytes from gray platelet syndrome patients
The Gray Platelet Syndrome (GPS) is a rare inherited bleeding disorder characterized by deficiency of platelet α-granules, macrothrombocytopenia and marrow fibrosis. The autosomal recessive form of GPS is linked to loss of function mutations in NBEAL2, which is predicted to regulate granule trafficking in megakaryocytes, the platelet progenitors. We report the first analysis of cultured megakar...
متن کاملConstitutively released adenosine diphosphate regulates proplatelet formation by human megakaryocytes.
BACKGROUND The interaction of adenosine diphosphate with its P2Y(1) and P2Y(12) receptors on platelets is important for platelet function. However, nothing is known about adenosine diphosphate and its function in human megakaryocytes. DESIGN AND METHODS We studied the role of adenosine diphosphate and P2Y receptors on proplatelet formation by human megakaryocytes in culture. RESULTS Megakar...
متن کاملImmune thrombocytopenia: antiplatelet autoantibodies inhibit proplatelet formation by megakaryocytes and impair platelet production in vitro.
Primary immune thrombocytopenia is an autoimmune disease mediated by antiplatelet autoantibodies that cause platelet destruction and suppression of platelet production. In vitro effects of autoantibodies on megakaryocyte production and maturation have been reported recently. However, the impact of these autoantibodies on crucial megakaryocyte functions, proplatelet formation and subsequent plat...
متن کاملCanonical Wnt signaling in megakaryocytes regulates proplatelet formation.
Wnt signaling is involved in numerous aspects of vertebrate development and homeostasis, including the formation and function of blood cells. Here, we show that canonical and noncanonical Wnt signaling pathways are present and functional in megakaryocytes (MKs), with several Wnt effectors displaying MK-restricted expression. Using the CHRF288-11 cell line as a model for human MKs, the canonical...
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ژورنال
عنوان ژورنال: Scientific Reports
سال: 2016
ISSN: 2045-2322
DOI: 10.1038/srep23213