4.6 Article

Matrix Remodeling in Pulmonary Fibrosis and Emphysema

出版社

AMER THORACIC SOC
DOI: 10.1165/rcmb.2015-0166PS

关键词

extracellular matrix; myofibroblasts; transforming growth factor-beta; pulmonary fibrosis; emphysema

资金

  1. National Institutes of Health (NIH) [P01 HL114470, R01 AG046210]
  2. NIH [HL102371]
  3. Veteren's Administration grant [I01BX001756]

向作者/读者索取更多资源

Pulmonary fibrosis and emphysema are chronic lung diseases characterized by a progressive decline in lung function, resulting in significant morbidity and mortality. A hallmark of these diseases is recurrent or persistent alveolar epithelial injury, typically caused by common environmental exposures such as cigarette smoke. We propose that critical determinants of the outcome of the injury-repair processes that result in fibrosis versus emphysema are mesenchymal cell fate and associated extracellular matrix dynamics. In this review, we explore the concept that regulation of mesenchymal cells under the influence of soluble factors, in particular transforming growth factor-beta 1, and the extracellular matrix determine the divergent tissue remodeling responses seen in pulmonary fibrosis and emphysema.

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