Defective AP-3-dependent VAMP8 trafficking impairs Weibel-Palade body exocytosis in Hermansky-Pudlak Syndrome type 2 blood outgrowth endothelial cells.
Journal
Haematologica
ISSN: 1592-8721
Titre abrégé: Haematologica
Pays: Italy
ID NLM: 0417435
Informations de publication
Date de publication:
10 2019
10 2019
Historique:
received:
07
10
2018
accepted:
09
01
2019
pubmed:
12
1
2019
medline:
8
7
2020
entrez:
12
1
2019
Statut:
ppublish
Résumé
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 bodies during maturation is dependent on the adaptor protein complex 3 complex. Mutations in the
Identifiants
pubmed: 30630984
pii: haematol.2018.207787
doi: 10.3324/haematol.2018.207787
pmc: PMC6886443
doi:
Substances chimiques
AP3B1 protein, human
0
Adaptor Protein Complex 3
0
Adaptor Protein Complex beta Subunits
0
R-SNARE Proteins
0
VAMP8 protein, human
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
2091-2099Informations de copyright
Copyright© 2019 Ferrata Storti Foundation.
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