Intracellular Storage and Regulated Secretion of Von Willebrand Factor in Quantitative Von Willebrand Disease

Several missense mutations in the von Willebrand Factor (VWF) gene of von Willebrand disease (VWD) patients have been shown to cause impaired constitutive secretion and intracellular retention of VWF. However, the effects of those mutations on the intracellular storage in Weibel-Palade bodies (WPBs)...

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Detalhes bibliográficos
Main Authors: Wang, Jiong-Wei, Valentijn, Karine M., de Boer, Hetty C., Dirven, Richard J., van Zonneveld, Anton Jan, Koster, Abraham J., Voorberg, Jan, Reitsma, Pieter H., Eikenboom, Jeroen
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Biochemistry and Molecular Biology 2011
Assuntos:
Molecular Bases of Disease
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3129199/
https://ncbi.nlm.nih.gov/pubmed/21596755
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M110.215194
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