Journal article
Fibulin-1c regulates transforming growth factor–β activation in pulmonary tissue fibrosis
G Liu, MA Cooley, AG Jarnicki, T Borghuis, PM Nair, G Tjin, AC Hsu, TJ Haw, M Fricker, CL Harrison, B Jones, NG Hansbro, PA Wark, JC Horvat, W Scott Argraves, BG Oliver, DA Knight, JK Burgess, PM Hansbro
Jci Insight | AMER SOC CLINICAL INVESTIGATION INC | Published : 2019
Open access
Abstract
Tissue remodeling/fibrosis is a major feature of all fibrotic diseases, including idiopathic pulmonary fibrosis (IPF). It is underpinned by accumulating extracellular matrix (ECM) proteins. Fibulin-1c (Fbln1c) is a matricellular ECM protein associated with lung fibrosis in both humans and mice and stabilizes collagen formation. Here we discovered that Fbln1c was increased in the lung tissues of patients with IPF and experimental bleomycin-induced pulmonary fibrosis. Fbln1c-deficient (Fbln1c–/–) mice had reduced pulmonary remodeling/fibrosis and improved lung function after bleomycin challenge. Fbln1c interacted with fibronectin, periostin, and tenascin-C in collagen deposits following bleomy..
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Awarded by Newcastle University
Funding Acknowledgements
This work was supported as follows. GL was supported by Lung Foundation Australia/Lizotte Family Research Award. The NIH supported WSA and MAC. AGJ was supported by Lung Foundation of Australia/Boehringer Ingelheim COPD Research Fellowship. BGO and JKB were supported by National Health and Medical Research Council (NHMRC) Career Development Fellowships. JKB was supported by a University of Groningen/European Union Rosalind Franklin Fellowship. PMH received fellowships and grants from NHMRC of Australia (NHMRC 1079187) and the Brawn Foundation, Faculty of Health and Medicine, the University of Newcastle. This work is dedicated to the memory of W. Scott Argraves, who passed away during the completion of this study.