Journal article
The matricellular protein Fibulin-1 is a better marker of disease progression than periostin, Tenascin-C and fibronectin in patients with idiopathic pulmonary fibrosis
Respirology, Vol.19, pp.32-34
2014
Abstract
Prediction of disease progression in idiopathic pulmonary fibrosis (IPF) remains challenging in the clinical setting. We have previously shown that the matricellular protein fibulin-1 enhances proliferation of mesenchymal cells. Increased levels of extracellular matrix (ECM) proteins may be a consequence of activated fibroblasts that produce excessive ECM proteins in the context of fibrotic disease.
The aim of this study was to compare the utility of the ECM proteins fibulin-1, periostin, tenascin-C and fibronectin as biomarkers of disease progression in patients with IPF.
Primary parenchymal fibroblasts derived from 8 patients with IPF and 7 subjects without lung disease were assessed for levels of cell-secreted ECM protein production. Serum and cell-secreted fibroblast proteins were measured by western blot (fibulin-1) and sandwich ELISA (periostin, tenascin-C, fibronectin) in 72 patients with IPF and 17 subjects without lung disease. Levels of the proteins were measured in the distal lung parenchyma of 20 patients with IPF and 5 subjects without lung disease using immunohistochemistry. Disease progression in patients with IPF was defined as a significant reduction in lung function or death within the first year of blood draw.
Fibulin-1 was the only of the 4 ECM proteins to accurately predict disease progression in patients with IPF (AUC 0.71, 95%CI 0.6 to 0.9, p = 0.01). Levels of fibulin-1 and periostin were increased in the serum and tissue of patients with IPF (p < 0.05) but only levels of fibulin-1 inversely correlated with lung function (r = −0.9, p < 0.05). In addition, only cell-secreted fibulin-1 production was increased in IPF fibroblasts compared to subjects without lung disease (p < 0.01).
The dysregulated levels of secreted ECM proteins can be reflected in the serum and act as biomarkers of activated fibroblasts and disease progression. Increased cell-secreted fibulin-1 production is an intrinsic quality of fibroblasts from patients with IPF and dysregulation was not seen in periostin or tenascin-C levels.
Details
- Title
- The matricellular protein Fibulin-1 is a better marker of disease progression than periostin, Tenascin-C and fibronectin in patients with idiopathic pulmonary fibrosis
- Authors/Creators
- J. Jaffar (Author/Creator)D. Van Ly (Author/Creator)L. Munk (Author/Creator)T. Corte (Author/Creator)S. Cerri (Author/Creator)L. Richeldi (Author/Creator)P. Wolters (Author/Creator)C. Prêle (Author/Creator)R. Oliver (Author/Creator)B. Oliver (Author/Creator)J. Black (Author/Creator)J. Burgess (Author/Creator)
- Publication Details
- Respirology, Vol.19, pp.32-34
- Publisher
- Wiley-Blackwell
- Identifiers
- 991005541769007891
- Copyright
- © 2014 The Authors/Asian Pacific Society of Respirology
- Murdoch Affiliation
- Murdoch University
- Language
- English
- Resource Type
- Journal article
- Additional Information
- Ann Woolcock Young Investigator Award - Oral Presentations of the Thoracic Society of Australia & New Zealand and the Australian & New Zealand Society of Respiratory Science 2014 Annual Scientific Meetings, 4‐9 April 2014, Adelaide Convention Centre, Adelaide, SA
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- Citation topics
- No Topic Assigned
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- Web Of Science research areas
- Respiratory System
- ESI research areas
- Clinical Medicine