Abstract
A set of lung diseases share the tendency for the development of progressive fibrosis ultimately leading to respiratory failure. This review examines the common pathogenetic features of these disorders in light of recent observations in both humans and animal models of disease, which reveal important pathways of lung matrix remodeling.
Publication types
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Research Support, U.S. Gov't, P.H.S.
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Review
MeSH terms
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Animals
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Collagen / chemistry
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Disease Models, Animal
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Disease Progression
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Epithelium / pathology
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Fibrosis
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Humans
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Inflammation
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Lung / metabolism*
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Lung Diseases / metabolism*
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Mice
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Models, Anatomic
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Models, Biological
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Mutation
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Pulmonary Fibrosis / pathology*
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Signal Transduction
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Transforming Growth Factor beta / genetics
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Transforming Growth Factor beta / metabolism
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Transforming Growth Factor beta1
Substances
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TGFB1 protein, human
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Tgfb1 protein, mouse
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Transforming Growth Factor beta
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Transforming Growth Factor beta1
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Collagen