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10.1371/journal.pone.0121994

http://scihub22266oqcxt.onion/10.1371/journal.pone.0121994
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suck abstract from ncbi


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pmid25803806      PLoS+One 2015 ; 10 (3): ä
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  • Regulatory Components of the Alternative Complement Pathway in Endothelial Cell Cytoplasm, Factor H and Factor I, Are Not Packaged in Weibel-Palade Bodies #MMPMID25803806
  • Turner NA; Sartain SE; Hui SK; Moake JL
  • PLoS One 2015[]; 10 (3): ä PMID25803806show ga
  • It was recently reported that factor H, a regulatory component of the alternative complement pathway, is stored with von Willebrand factor (VWF) in the Weibel-Palade bodies of endothelial cells. If this were to be the case, it would have therapeutic importance for patients with the atypical hemolytic-uremic syndrome that can be caused either by a heterozygous defect in the factor H gene or by the presence of an autoantibody against factor H. The in vivo Weibel-Palade body secretagogue, des-amino-D-arginine vasopressin (DDAVP), would be expected to increase transiently the circulating factor H levels, in addition to increasing the circulating levels of VWF. We describe experiments demonstrating that factor H is released from endothelial cell cytoplasm without a secondary storage site. These experiments showed that factor H is not stored with VWF in endothelial cell Weibel-Palade bodies, and is not secreted in response in vitro in response to the Weibel-Palade body secretagogue, histamine. Furthermore, the in vivo Weibel-Palade body secretagogue, DDAVP does not increase the circulating factor H levels concomitantly with DDAVP-induced increased VWF. Factor I, a regulatory component of the alternative complement pathway that is functionally related to factor H, is also located in endothelial cell cytoplasm, and is also not present in endothelial cell Weibel-Palade bodies. Our data demonstrate that the factor H and factor I regulatory proteins of the alternative complement pathway are not stored in Weibel-Palade bodies. DDAVP induces the secretion into human plasma of VWF ?- but not factor H.
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