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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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Detalhes bibliográficos
Publicado no:PLoS One
Main Authors: 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: Artigo
Idioma:Inglês
Publicado em: Public Library of Science 2016
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4961407/
https://ncbi.nlm.nih.gov/pubmed/27459687
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0159177
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