BLOC-2 subunit HPS6 deficiency affects the tubulation and secretion of von Willebrand factor from mouse endothelial cells

Hermansky-Pudlak syndrome (HPS) is a recessive disorder with bleeding diathesis, which has been linked to platelet granule defects. Both platelet granules and endothelial Weibel-Palade bodies (WPBs) are members of lysosome-related organelles (LROs) whose formation is regulated by HPS protein associa...

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Detalles Bibliográficos
Autores principales: Ma, Jing, Zhang, Zhe, Yang, Lin, Kriston-Vizi, Janos, Cutler, Daniel F., Li, Wei
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Science press ;, Elsevier 2016
Materias:
Original Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5199771/
https://www.ncbi.nlm.nih.gov/pubmed/27889498
http://dx.doi.org/10.1016/j.jgg.2016.09.007

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5199771/
https://www.ncbi.nlm.nih.gov/pubmed/27889498
http://dx.doi.org/10.1016/j.jgg.2016.09.007

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