ADAMTS18 Deficiency Leads to Pulmonary Hypoplasia and Bronchial Microfibril Accumulation

被引：16

作者：

Lu, Tiantian ^{[1
]}

Lin, Xiaotian ^{[1
]}

Pan, Yi-Hsuan ^{[1
]}

Yang, Ning ^{[1
]}

Ye, Shuai ^{[1
]}

Zhang, Qi ^{[1
]}

Wang, Caiyun ^{[1
]}

Zhu, Rui ^{[1
]}

Zhang, Tianhao ^{[1
]}

Wisniewski, Thomas M. ^{[2
,3
,4
]}

Cao, Zhongwei ^{[5
,6
]}

Ding, Bi-Sen ^{[5
,6
]}

Dang, Suying ^{[7
]}

Zhang, Wei ^{[1
]}

机构：

[1] East China Normal Univ, Sch Life Sci, Minist Educ & Shanghai, Key Lab Brain Funct Genom, 3663 North Zhongshan Rd, Shanghai 200062, Peoples R China

[2] NYU, Sch Med, Dept Neurol, New York, NY 10016 USA

[3] NYU, Sch Med, Dept Pathol, New York, NY 10016 USA

[4] NYU, Sch Med, Dept Psychiat, New York, NY 10016 USA

[5] Sichuan Univ, West China Univ Hosp 2, State Key Lab Biotherapy, Key Lab Birth Defects & Related Dis Women & Child, Chengdu, Peoples R China

[6] Collaborat Innovat Ctr Biotherapy, Chengdu, Peoples R China

[7] Shanghai Jiao Tong Univ, Sch Med, Dept Biochem & Mol Cell Biol, 227 South Chongqing Rd, Shanghai 200025, Peoples R China

来源：

ISCIENCE | 2020年 / 23卷 / 09期

基金：

中国国家自然科学基金;

关键词：

EXTRACELLULAR-MATRIX; MOUSE MODEL; FIBRILLIN-1; EXPRESSION; PROMOTES; LUNG; METALLOPROTEASE; IDENTIFICATION; SUPERFAMILY; DISINTEGRIN;

D O I：

10.1016/j.isci.2020.101472

中图分类号：

O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

ADAMTSs (a disintegrin and metalloproteinase with thrombospondin motifs) are secreted metalloproteinases that play a major role in the assembly and degradation of the extracellular matrix (ECM). In this study, we show that ADAMTS18, produced by the epithelial cells of distal airways and mesenchymal cells in lung apex at early embryonic stages, serves as a morphogen in lung development. ADAMTS18 deficiency leads to reduced number and length of bronchi, tipped lung apexes, and dilated alveoli. These developmental defects worsen lipopolysaccharide-induced acute lung injury and bleomycin-induced lung fibrosis in adult Adamts18-deficient mice. ADAMTS18 deficiency also causes increased levels of fibrillin1 and fibrillin2, bronchial microfibril accumulation, decreased focal adhesion kinase signaling, and disruption of F-actin organization. Our findings indicate that ECM homeostasis mediated by ADAMTS18 is pivotal in airway branching morphogenesis.

引用

页数：33

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