Hematology education. European Hematology Association. Congress. Education Program最新文献

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Lessons Learned from Animal Models of Inherited Bleeding Disorders. 遗传性出血性疾病动物模型的经验教训。
Timothy C Nichols
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引用次数: 0
Down-regulation of the clotting cascade by the protein C pathway. 蛋白C途径对凝血级联的下调。
Fabian Stavenuiter, Eveline A M Bouwens, Laurent O Mosnier
{"title":"Down-regulation of the clotting cascade by the protein C pathway.","authors":"Fabian Stavenuiter,&nbsp;Eveline A M Bouwens,&nbsp;Laurent O Mosnier","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>The protein C pathway provides important biological activities to maintain the fluidity of the circulation, prevent thrombosis, and protect the integrity of the vasculature in response to injury. Activated protein C (APC), in concert with its cofactors and cell receptors, assembles in specific macromolecular complexes to provide efficient proteolysis of multiple substrates that result in anticoagulant and cytoprotective activities. Numerous studies on APC's structure-function relation with its cofactors, cell receptors, and substrates provide valuable insights into the molecular mechanisms and presumed assembly of the macromolecular complexes that are responsible for APC's activities. These insights allow for molecular engineering approaches specifically targeting the interaction of APC with one of its substrates or cofactors. Thus far, these approaches resulted in several anticoagulant-selective and cytoprotective-selective APC mutants, which provide unique insights into the relative contributions of APC's anticoagulant or cytoprotective activities to the beneficial effects of APC in various murine injury and disease models. Because of its multiple physiological and pharmacological activities, the anticoagulant and cytoprotective protein C pathway have important implications for the (patho)physiology of vascular disease and for translational research exploring novel therapeutic strategies to combat complex medical disorders such as thrombosis, inflammation, ischemic stroke and neurodegenerative disease.</p>","PeriodicalId":90175,"journal":{"name":"Hematology education. European Hematology Association. Congress. Education Program","volume":"7 1","pages":"365-374"},"PeriodicalIF":0.0,"publicationDate":"2013-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3985519/pdf/nihms513449.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32270199","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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