Alveolar Macrophage Dysregulation in Hermansky-Pudlak Syndrome Type 1

Rationale: Individuals with Hermansky-Pudlak syndrome type 1 (HPS-1), an autosomal recessive disorder characterized by defective biogenesis of lysosome-related organelles, develop an accelerated form of progressive fibrotic lung disease. The etiology of pulmonary fibrosis associated with HPS-1 is un...

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Main Authors: Rouhani, Farshid N., Brantly, Mark L., Markello, Thomas C., Helip-Wooley, Amanda, O'Brien, Kevin, Hess, Richard, Huizing, Marjan, Gahl, William A., Gochuico, Bernadette R.
Formato: Artigo
Idioma:Inglês
Publicado: American Thoracic Society 2009
Assuntos:
F. Interstitial Lung Disease
Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC2784416/
https://ncbi.nlm.nih.gov/pubmed/19729668
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1164/rccm.200901-0023OC
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