Immunophenotypic and Ultrastructural Analysis of Mast Cells in Hermansky-Pudlak Syndrome Type-1: A Possible Connection to Pulmonary Fibrosis

Hermansky-Pudlak Syndrome type-1 (HPS-1) is an autosomal recessive disorder caused by mutations in HPS1 which result in reduced expression of the HPS-1 protein, defective lysosome-related organelle (LRO) transport and absence of platelet delta granules. Patients with HPS-1 exhibit oculocutaneous alb...

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Detalles Bibliográficos
Autores principales: Kirshenbaum, Arnold S., Cruse, Glenn, Desai, Avanti, Bandara, Geethani, Leerkes, Maarten, Lee, Chyi-Chia R., Fischer, Elizabeth R., O’Brien, Kevin J., Gochuico, Bernadette R., Stone, Kelly, Gahl, William A., Metcalfe, Dean D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2016
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4961407/
https://www.ncbi.nlm.nih.gov/pubmed/27459687
http://dx.doi.org/10.1371/journal.pone.0159177

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