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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Autores principales: 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
Lenguaje:Inglês
Publicado: American Society for Biochemistry and Molecular Biology 2011
Materias:
Molecular Bases of Disease
Acceso en línea: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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