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The Alveolar Epithelium Determines Susceptibility to Lung Fibrosis in Hermansky-Pudlak Syndrome

Rationale: Hermansky-Pudlak syndrome (HPS) is a family of recessive disorders of intracellular trafficking defects that are associated with highly penetrant pulmonary fibrosis. Naturally occurring HPS mice reliably model important features of the human disease, including constitutive alveolar macrop...

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Detalhes bibliográficos
Main Authors: Young, Lisa R., Gulleman, Peter M., Bridges, James P., Weaver, Timothy E., Deutsch, Gail H., Blackwell, Timothy S., McCormack, Francis X.
Formato: Artigo
Idioma:Inglês
Publicado em: American Thoracic Society 2012
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3530211/
https://ncbi.nlm.nih.gov/pubmed/23043085
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1164/rccm.201207-1206OC
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