Fibulin-1 predicts disease progression in patients with idiopathic pulmonary fibrosis.
Jaffar, J
Unger, S
Corte, TJ
Keller, M
Wolters, PJ
Richeldi, L
Cerri, S
Prêle, CM
Hansbro, PM
Argraves, WS
Oliver, RA
Oliver, BG
Black, JL
Burgess, JK
- Publisher:
- American College of Chest Physicans
- Publication Type:
- Journal Article
- Citation:
- Chest, 2014, 146, (4), pp. 1055-1063
- Issue Date:
- 2014-01
Closed Access
Filename | Description | Size | |||
---|---|---|---|---|---|
Fibulin-1 predicts disease progression in patients wth idiopathic pulmonary fibrosis.pdf | Published version | 1.02 MB | Adobe PDF |
Copyright Clearance Process
- Recently Added
- In Progress
- Closed Access
This item is closed access and not available.
Full metadata record
Field | Value | Language |
---|---|---|
dc.contributor.author | Jaffar, J | |
dc.contributor.author | Unger, S | |
dc.contributor.author | Corte, TJ | |
dc.contributor.author | Keller, M | |
dc.contributor.author | Wolters, PJ | |
dc.contributor.author | Richeldi, L | |
dc.contributor.author | Cerri, S | |
dc.contributor.author | Prêle, CM | |
dc.contributor.author | Hansbro, PM | |
dc.contributor.author | Argraves, WS | |
dc.contributor.author | Oliver, RA | |
dc.contributor.author | Oliver, BG | |
dc.contributor.author | Black, JL | |
dc.contributor.author | Burgess, JK | |
dc.date.accessioned | 2021-03-03T23:19:52Z | |
dc.date.available | 2021-03-03T23:19:52Z | |
dc.date.issued | 2014-01 | |
dc.identifier.citation | Chest, 2014, 146, (4), pp. 1055-1063 | |
dc.identifier.issn | 0012-3692 | |
dc.identifier.issn | 1931-3543 | |
dc.identifier.uri | http://hdl.handle.net/10453/146731 | |
dc.description.abstract | Background The underlying mechanisms of idiopathic pulmonary fibrosis (IPF) are unknown. This progressive disease has high mortality rates and current models for prediction of mortality have limited value in identifying which patients will progress. We previously showed that the glycoprotein fibulin-1 is involved in enhanced proliferation and wound repair by mesenchymal cells, thus may contribute to lung fibrosis in IPF. Methods Serum, lung tissue and lung function values were obtained from four independent locations (Sydney and Perth, Australia, San Francisco, USA and Modena, Italy). Patients with IPF were followed for a minimum of one year and progression was defined as a significant decline in lung function or death. Primary parenchymal lung fibroblasts of 15 patients with and without IPF were cultured under non-stimulatory conditions. Fibulin-1 levels in serum and secreted or deposited by fibroblasts were measured by western blot and in lung tissue by immunohistochemistry. Results Serum fibulin-1 levels were increased in patients with IPF compared to subjects without lung disease (p=0.006). Furthermore, tissue fibulin-1 levels were increased in patients with IPF (p=0.02) and correlated negatively with lung function (r=-0.9, p<0.05). Primary parenchymal fibroblasts from patients with IPF produced more fibulin-1 than those from subjects without IPF (p<0.05). Finally, serum fibulin-1 levels at first blood draw predicted disease progression in IPF within 1 year (AUC 0.71, 95%CI 0.57 0.86, p=0.012). Conclusions Fibulin-1 is a novel potential biomarker for disease progression IPF and raise the possibility that it could be used as a target for the development of new treatments. | |
dc.format | ||
dc.language | eng | |
dc.publisher | American College of Chest Physicans | |
dc.relation | http://purl.org/au-research/grants/nhmrc/APP1026880 | |
dc.relation.ispartof | Chest | |
dc.relation.isbasedon | 10.1378/chest.13-2688 | |
dc.rights | info:eu-repo/semantics/closedAccess | |
dc.subject | 1103 Clinical Sciences | |
dc.subject.classification | Respiratory System | |
dc.subject.mesh | Fibroblasts | |
dc.subject.mesh | Humans | |
dc.subject.mesh | Disease Progression | |
dc.subject.mesh | Calcium-Binding Proteins | |
dc.subject.mesh | Blotting, Western | |
dc.subject.mesh | Cell Culture Techniques | |
dc.subject.mesh | Immunohistochemistry | |
dc.subject.mesh | Follow-Up Studies | |
dc.subject.mesh | Aged | |
dc.subject.mesh | Aged, 80 and over | |
dc.subject.mesh | Middle Aged | |
dc.subject.mesh | United States | |
dc.subject.mesh | Australia | |
dc.subject.mesh | Italy | |
dc.subject.mesh | Female | |
dc.subject.mesh | Male | |
dc.subject.mesh | Idiopathic Pulmonary Fibrosis | |
dc.subject.mesh | Biomarkers | |
dc.subject.mesh | Aged | |
dc.subject.mesh | Aged, 80 and over | |
dc.subject.mesh | Australia | |
dc.subject.mesh | Biomarkers | |
dc.subject.mesh | Blotting, Western | |
dc.subject.mesh | Calcium-Binding Proteins | |
dc.subject.mesh | Cell Culture Techniques | |
dc.subject.mesh | Disease Progression | |
dc.subject.mesh | Female | |
dc.subject.mesh | Fibroblasts | |
dc.subject.mesh | Follow-Up Studies | |
dc.subject.mesh | Humans | |
dc.subject.mesh | Idiopathic Pulmonary Fibrosis | |
dc.subject.mesh | Immunohistochemistry | |
dc.subject.mesh | Italy | |
dc.subject.mesh | Male | |
dc.subject.mesh | Middle Aged | |
dc.subject.mesh | United States | |
dc.title | Fibulin-1 predicts disease progression in patients with idiopathic pulmonary fibrosis. | |
dc.type | Journal Article | |
utslib.citation.volume | 146 | |
utslib.location.activity | United States | |
utslib.for | 1103 Clinical Sciences | |
utslib.for | 1103 Clinical Sciences | |
pubs.organisational-group | /University of Technology Sydney/Faculty of Science | |
pubs.organisational-group | /University of Technology Sydney/Strength - CHT - Health Technologies | |
pubs.organisational-group | /University of Technology Sydney/Faculty of Science/School of Life Sciences | |
pubs.organisational-group | /University of Technology Sydney | |
utslib.copyright.status | closed_access | * |
pubs.consider-herdc | false | |
dc.date.updated | 2021-03-03T23:19:50Z | |
pubs.issue | 4 | |
pubs.publication-status | Published | |
pubs.volume | 146 | |
utslib.citation.issue | 4 |
Abstract:
Background The underlying mechanisms of idiopathic pulmonary fibrosis (IPF) are unknown. This progressive disease has high mortality rates and current models for prediction of mortality have limited value in identifying which patients will progress. We previously showed that the glycoprotein fibulin-1 is involved in enhanced proliferation and wound repair by mesenchymal cells, thus may contribute to lung fibrosis in IPF. Methods Serum, lung tissue and lung function values were obtained from four independent locations (Sydney and Perth, Australia, San Francisco, USA and Modena, Italy). Patients with IPF were followed for a minimum of one year and progression was defined as a significant decline in lung function or death. Primary parenchymal lung fibroblasts of 15 patients with and without IPF were cultured under non-stimulatory conditions. Fibulin-1 levels in serum and secreted or deposited by fibroblasts were measured by western blot and in lung tissue by immunohistochemistry. Results Serum fibulin-1 levels were increased in patients with IPF compared to subjects without lung disease (p=0.006). Furthermore, tissue fibulin-1 levels were increased in patients with IPF (p=0.02) and correlated negatively with lung function (r=-0.9, p<0.05). Primary parenchymal fibroblasts from patients with IPF produced more fibulin-1 than those from subjects without IPF (p<0.05). Finally, serum fibulin-1 levels at first blood draw predicted disease progression in IPF within 1 year (AUC 0.71, 95%CI 0.57 0.86, p=0.012). Conclusions Fibulin-1 is a novel potential biomarker for disease progression IPF and raise the possibility that it could be used as a target for the development of new treatments.
Please use this identifier to cite or link to this item:
Download statistics for the last 12 months
Not enough data to produce graph