Myofibroblast differentiation and enhanced TGF-B signaling in cystic fibrosis lung disease

TGF-β, a mediator of pulmonary fibrosis, is a genetic modifier of CF respiratory deterioration. The mechanistic relationship between TGF-β signaling and CF lung disease has not been determined. To investigate myofibroblast differentiation in CF lung tissue as a novel pathway by which TGF-β signaling...

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Bibliographic Details
Published in:PloS one 2013-08, Vol.8 (8), p.e70196-e70196
Main Authors: Harris, William T, Kelly, David R, Zhou, Yong, Wang, Dezhi, MacEwen, Mark, Macewen, Mark, Hagood, James S, Clancy, J P, Ambalavanan, Namasivayam, Sorscher, Eric J
Format: Article
Language:English
Subjects:
Actin
Adult
Analysis
Automation
Biology
Bone morphogenetic proteins
Cell Differentiation
Children & youth
Collagen
Cystic fibrosis
Cystic Fibrosis - metabolism
Cystic Fibrosis - pathology
Differentiation
Female
Fibroblasts
Fibrosis
Growth factors
Humans
Hypoxia
Idiopathic Pulmonary Fibrosis - metabolism
Idiopathic Pulmonary Fibrosis - pathology
Inflammation
Lung - metabolism
Lung - pathology
Lung diseases
Lungs
Male
Mathematics
Measurement techniques
Medical research
Medicine
Middle Aged
Models, Biological
Muscle proteins
Muscles
Myofibroblasts - cytology
Myofibroblasts - metabolism
Pediatrics
Phosphorylation
Pulmonary fibrosis
Quantitative analysis
Respiratory tract
Sampling methods
Signal Transduction
Signaling
Smad2 protein
Smad2 Protein - metabolism
Smooth muscle
Studies
Transforming Growth Factor beta - metabolism
Transforming Growth Factor beta1 - metabolism
Transforming growth factor-b
Transforming growth factor-b1
Transforming growth factors
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Summary:TGF-β, a mediator of pulmonary fibrosis, is a genetic modifier of CF respiratory deterioration. The mechanistic relationship between TGF-β signaling and CF lung disease has not been determined. To investigate myofibroblast differentiation in CF lung tissue as a novel pathway by which TGF-β signaling may contribute to pulmonary decline, airway remodeling and tissue fibrosis. Lung samples from CF and non-CF subjects were analyzed morphometrically for total TGF-β1, TGF-β signaling (Smad2 phosphorylation), myofibroblast differentiation (α-smooth muscle actin), and collagen deposition (Masson trichrome stain). TGF-β signaling and fibrosis are markedly increased in CF (p
ISSN:1932-6203
1932-6203
DOI:10.1371/journal.pone.0070196