| Literature DB >> 22951324 |
Christophe Goubau1, Gunnar M Buyse, Michela Di Michele, Chris Van Geet, Kathleen Freson.
Abstract
Platelet function in primary hemostasis involves the secretion of granules upon activation, providing the localized delivery of effector proteins at sites of vascular injury. The sequential process of regulated secretion in platelets, from the biogenesis of the granules, through their transport and up to the exocytotic fusion process at the acceptor membrane, involves a complex molecular machinery conserved between some other specialized cells such as neurons. Mutations in genes encoding proteins involved in this process of granule trafficking have helped towards demystification of the underlying secretory mechanisms. Human diseases of trafficking encompass a broad symptomatology including a platelet-related bleeding diathesis and neuronal problems. In this review, we want to highlight the similarities in granule biology between platelets and neurons and further focus on some granule trafficking disorders that result in bleeding and neuropathology. This review provides evidence that platelet research can be expanded from traditional studies of isolated thrombopathies to the field of neuropathologies that include a platelet secretion defect.Entities:
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Year: 2012 PMID: 22951324 DOI: 10.1016/j.ejpn.2012.08.005
Source DB: PubMed Journal: Eur J Paediatr Neurol ISSN: 1090-3798 Impact factor: 3.140