Gray platelet syndrome and defective thrombo-inflammation in Nbeal2-deficient mice
Journal of Clinical Investigation, ISSN: 0021-9738, Vol: 123, Issue: 8, Page: 3331-3342
2013
- 158Citations
- 112Captures
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Example: if you select the 1-year option for an article published in 2019 and a metric category shows 90%, that means that the article or review is performing better than 90% of the other articles/reviews published in that journal in 2019. If you select the 3-year option for the same article published in 2019 and the metric category shows 90%, that means that the article or review is performing better than 90% of the other articles/reviews published in that journal in 2019, 2018 and 2017.
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Metrics Details
- Citations158
- Citation Indexes157
- 157
- CrossRef138
- Patent Family Citations1
- Patent Families1
- Captures112
- Readers112
- 112
Article Description
Platelets are anuclear organelle-rich cell fragments derived from bone marrow megakaryocytes (MKs) that safeguard vascular integrity. The major platelet organelles, α-granules, release proteins that participate in thrombus formation and hemostasis. Proteins stored in α-granules are also thought to play a role in inflammation and wound healing, but their functional significance in vivo is unknown. Mutations in NBEAL2 have been linked to gray platelet syndrome (GPS), a rare bleeding disorder characterized by macrothrombocytopenia, with platelets lacking α-granules. Here we show that Nbeal2-knockout mice display the characteristics of human GPS, with defective α-granule biogenesis in MKs and their absence from platelets. Nbeal2 deficiency did not affect MK differentiation and proplatelet formation in vitro or platelet life span in vivo. Nbeal2-deficient platelets displayed impaired adhesion, aggregation, and coagulant activity ex vivo that translated into defective arterial thrombus formation and protection from thrombo-inflammatory brain infarction following focal cerebral ischemia. In a model of excisional skin wound repair, Nbeal2-deficient mice exhibited impaired development of functional granulation tissue due to severely reduced differentiation of myofibroblasts in the absence of α-granule secretion. This study demonstrates that platelet α-granule constituents are critically required not only for hemostasis but also thrombosis, acute thrombo-inflammatory disease states, and tissue reconstitution after injury.
Bibliographic Details
http://www.scopus.com/inward/record.url?partnerID=HzOxMe3b&scp=84881254041&origin=inward; http://dx.doi.org/10.1172/jci69210; http://www.ncbi.nlm.nih.gov/pubmed/23863626; http://www.jci.org/articles/view/69210#sd; http://dx.doi.org/10.1172/jci69210ds1; http://www.jci.org/articles/view/69210; https://dx.doi.org/10.1172/jci69210; https://www.jci.org/articles/view/69210
American Society for Clinical Investigation
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