Fibulin-1c regulates transforming growth factor-β activation in pulmonary tissue fibrosis

被引：66

作者：

Liu, Gang ^{[1
,2
,3
,4
]}

Cooley, Marion A. ^{[5
]}

Jarnicki, Andrew G. ^{[1
,2
,6
]}

Borghuis, Theo ^{[7
]}

Nair, Prema M. ^{[1
,2
]}

Tjin, Gavin ^{[8
]}

Hsu, Alan C. ^{[1
,2
]}

Haw, Tatt Jhong ^{[1
,2
]}

Fricker, Michael ^{[1
,2
]}

Harrison, Celeste L. ^{[1
,2
]}

Jones, Bernadette ^{[1
,2
]}

Hansbro, Nicole G. ^{[1
,2
,3
,4
]}

Wark, Peter A. ^{[1
,2
]}

Horvat, Jay C. ^{[1
,2
]}

Argraves, W. Scott ^{[9
]}

Oliver, Brian G. ^{[3
,8
]}

Knight, Darryl A. ^{[1
,2
]}

Burgess, Janette K. ^{[7
,8
]}

Hansbro, Philip M. ^{[1
,2
,3
,4
]}

机构：

[1] Hunter Med Res Inst, Prior Res Ctr Hlth Lungs, Newcastle, NSW, Australia

[2] Univ Newcastle, Newcastle, NSW, Australia

[3] Univ Technol Sydney, Sch Life Sci, Sydney, NSW, Australia

[4] Centenary Inst, Sydney, NSW, Australia

[5] Augusta Univ, Dept Oral Biol & Diagnost Sci, Augusta, GA USA

[6] Univ Melbourne, Dept Pharmacol & Therapeut, Parkville, Vic, Australia

[7] Univ Groningen, Groningen Res Inst Asthma, Univ Med Ctr Groningen, Groningen Res Inst Asthma & COPD,Dept Pathol & Me, Groningen, Netherlands

[8] Univ Sydney, Woolcock Inst Med Res, Discipline Pharmacol, Sydney, NSW, Australia

[9] Med Univ South Carolina, Dept Regenerat Med & Cell Biol, Charleston, SC 29425 USA

来源：

JCI INSIGHT | 2019年 / 4卷 / 16期

基金：

英国医学研究理事会; 澳大利亚国家健康与医学研究理事会;

关键词：

ALLERGIC AIRWAYS DISEASE; EXTRACELLULAR-MATRIX; FEEDBACK LOOP; KEY FEATURES; PROTEIN; EXPRESSION; METALLOPROTEINASES; INFLAMMATION; CONTRIBUTES; INFECTION;

D O I：

10.1172/jci.insight.124529

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

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. FbInic interacted with fibronectin, periostin, and tenascin-C in collagen deposits following bleomycin challenge. In a potentially novel mechanism of fibrosis, FbInic bound to latent TGF-beta-binding protein 1(LTBP1) to induce TGF-beta activation and mediated downstream Smad3 phosphorylation/signaling. This process increased myofibroblast numbers and collagen deposition. Fbln1c and LTBP1 colocalized in lung tissues from patients with IPF. Thus, Fbln1c may be a novel driver of TGF-beta-induced fibrosis involving LTBP1 and may be an upstream therapeutic target.

引用

页数：17

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