| First Author | Liu G | Year | 2016 |
| Journal | JCI Insight | Volume | 1 |
| Issue | 9 | PubMed ID | 27398409 |
| Mgi Jnum | J:263505 | Mgi Id | MGI:6164428 |
| Doi | 10.1172/jci.insight.86380 | Citation | Liu G, et al. (2016) Fibulin-1 regulates the pathogenesis of tissue remodeling in respiratory diseases. JCI Insight 1(9):e86380 |
| abstractText | 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-alpha, 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. |
