Fibulin-1 regulates the pathogenesis of tissue remodeling in respiratory diseases Academic Article uri icon

  • Overview
  • Research
  • Identity
  • Additional Document Info
  • View All


  • Airway and/or lung remodeling, involving exaggerated extracellular matrix (ECM) protein deposition, is a critical feature common to pulmonary diseases including chronic obstructive pulmonary disease (COPD), asthma, and idiopathic pulmonary fibrosis (IPF). Fibulin-1 (Fbln1), an important ECM protein involved in matrix organization, may be involved in the pathogenesis of these diseases. We found that Fbln1 was increased in COPD patients and in cigarette smoke-induced (CS-induced) experimental COPD in mice. Genetic or therapeutic inhibition of Fbln1c protected against CS-induced airway fibrosis and emphysema-like alveolar enlargement. In experimental COPD, this occurred through disrupted collagen organization and interactions with fibronectin, periostin, and tenascin-c. Genetic inhibition of Fbln1c also reduced levels of pulmonary inflammatory cells and proinflammatory cytokines/chemokines (TNF-α, IL-33, and CXCL1) in experimental COPD. Fbln1c-/- mice also had reduced airway remodeling in experimental chronic asthma and pulmonary fibrosis. Our data show that Fbln1c may be a therapeutic target in chronic respiratory diseases.


  • Liu, Gang
  • Cooley, Marion A
  • Jarnicki, Andrew G
  • Hsu, Alan C-Y
  • Nair, Prema M
  • Haw, Tatt Jhong
  • Fricker, Michael
  • Gellatly, Shaan L
  • Kim, Richard Y
  • Inman, Mark David
  • Tjin, Gavin
  • Wark, Peter AB
  • Walker, Marjorie M
  • Horvat, Jay C
  • Oliver, Brian G
  • Argraves, W Scott
  • Knight, Darryl A
  • Burgess, Janette K
  • Hansbro, Philip M

publication date

  • June 16, 2016