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J Exp Med. 2011 Jul 4;208(7):1339-50. doi: 10.1084/jem.20110551.

Integrating mechanisms of pulmonary fibrosis.

Author information

1
Program in Barrier Immunity and Repair and the Immunopathogenesis Section, Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA. twynn@niaid.nih.gov

Abstract

Pulmonary fibrosis is a highly heterogeneous and lethal pathological process with limited therapeutic options. Although research on the pathogenesis of pulmonary fibrosis has frequently focused on the mechanisms that regulate the proliferation, activation, and differentiation of collagen-secreting myofibroblasts, recent studies have identified new pathogenic mechanisms that are critically involved in the initiation and progression of fibrosis in a variety of settings. A more detailed and integrated understanding of the cellular and molecular mechanisms of pulmonary fibrosis could help pave the way for effective therapeutics for this devastating and complex disease.

PMID:
21727191
PMCID:
PMC3136685
DOI:
10.1084/jem.20110551
[Indexed for MEDLINE]
Free PMC Article

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