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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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| Auteurs principaux: | , , , , , , , , |
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| Format: | Artigo |
| Langue: | Inglês |
| Publié: |
American Thoracic Society
2009
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| Sujets: | |
| Accès en ligne: | 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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