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Article

The alveolar epithelium determines susceptibility to lung fibrosis in Hermansky-Pudlak syndrome.

American journal of respiratory and critical care medicine - 15 Nov 2012

Young Lisa R, Gulleman Peter M, Bridges James P, Weaver Timothy E, Deutsch Gail H, Blackwell Timothy S, McCormack Francis X

Abstract excerpt

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 macrophage activation and susceptibility to profibrotic stimuli. OBJECTIVES: To decipher which cell lineage(s) in the...

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Identifiers

PMID: 23043085
DOI: 10.1164/rccm.201207-1206OC
PMCID: PMC3530211

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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