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Defective AP-3-dependent VAMP8 trafficking impairs Weibel-Palade body exocytosis in Hermansky-Pudlak Syndrome type 2 blood outgrowth endothelial cells

Weibel-Palade bodies are endothelial secretory organelles that contain von Willebrand factor, P-selectin and CD63. Release of von Willebrand factor from Weibel-Palade bodies is crucial for platelet adhesion during primary hemostasis. Endosomal trafficking of proteins like CD63 to Weibel-Palade bodie...

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Detalles Bibliográficos
Autores principales: Karampini, Ellie, Schillemans, Maaike, Hofman, Menno, van Alphen, Floris, de Boer, Martin, Kuijpers, Taco W., van den Biggelaar, Maartje, Voorberg, Jan, Bierings, Ruben
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Ferrata Storti Foundation 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6886443/
https://www.ncbi.nlm.nih.gov/pubmed/30630984
http://dx.doi.org/10.3324/haematol.2018.207787