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Morty, R.E.* ; Königshoff, M. ; Eickelberg, O.

Transforming growth factor-β signaling across ages: From distorted lung development to chronic obstructive pulmonary disease.

Ann. Am. Thorac. Soc. 6, 607-613 (2009)
DOI PMC
Open Access Green as soon as Postprint is submitted to ZB.
The transforming growth factor (TGF)-beta superfamily of secreted growth factors consists of more than 40 members, including the TGF-beta isoforms themselves, bone morphogenetic proteins, and activins. Most of these factors have been shown to be essential for proper organ development, a process often recapitulated in chronic diseases. Importantly, TGF-beta superfamily members are key regulators of extracellular matrix composition and alveolar epithelial cell and fibroblast function in the lung. Both during lung development and disease, TGF-betas therefore control lung homeostasis by providing the structural requirements and functional micromilieu needed for physiological epithelial cell function and proper gas exchange. Prolonged alterations of TGF-beta signaling have been shown to result in structural changes in the lung that compromise gas exchange and lung function, as seen in arrested lung development, a feature of bronchopulmonary dysplasia, lung fibrosis, and chronic obstructive pulmonary disease. All these syndromes share a loss of functional alveolar structures, which ultimately leads to a decreased life expectancy. In this review, we cover our current understanding of the impact of TGF-beta signaling on chronic lung disease. We focus on distorted TGF-beta signaling in bronchopulmonary dysplasia and chronic obstructive pulmonary disease as prototype diseases of the premature and matured lung, respectively, which are both characterized by functional and structural loss of alveolar units.
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Publication type Article: Journal article
Document type Scientific Article
Corresponding Author
Keywords bronchopulmonary dysplasia; chronic obstructive pulmonary disease; emphysema; small airway disease; transforming growth factor-β
ISSN (print) / ISBN 2329-6933
e-ISSN 2325-6621
Journal Annals of the American Thoracic Society
Quellenangaben Volume: 6, Issue: 7, Pages: 607-613 Article Number: , Supplement: ,
Publisher American Thoracic Society
Publishing Place New York, NY
Non-patent literature Publications
Reviewing status Peer reviewed
Institute(s) Institute of Lung Biology (LHI)