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J Exp Med. 2015 Feb 9;212(2):235-52. doi: 10.1084/jem.20121878. Epub 2015 Jan 12.

Blocking follistatin-like 1 attenuates bleomycin-induced pulmonary fibrosis in mice.

Author information

1
State Key Laboratory of Medicinal Chemical Biology, College of Life Sciences, Nankai University, Tianjin 300071, China Cam-Su Genomic Resource Center, Soochow University, Suzhou 215123, China.
2
State Key Laboratory of Medicinal Chemical Biology, College of Life Sciences, Nankai University, Tianjin 300071, China School of Pharmaceutical Science, Jiangnan University, Wuxi 214122, China.
3
State Key Laboratory of Medicinal Chemical Biology, College of Life Sciences, Nankai University, Tianjin 300071, China Respiratory Department, Tianjin Medical University General Hospital, Tianjin 300052, China.
4
State Key Laboratory of Medicinal Chemical Biology, College of Life Sciences, Nankai University, Tianjin 300071, China.
5
State Key Laboratory of Biomembrane and Membrane Biotechnology, School of Life Sciences, Tsinghua University, Beijing 100084, China.
6
Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA 90048.
7
Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100020, China.
8
Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA 90048 ningwen108@nankai.edu.cn dianhua.jiang@cshs.org.
9
State Key Laboratory of Medicinal Chemical Biology, College of Life Sciences, Nankai University, Tianjin 300071, China ningwen108@nankai.edu.cn dianhua.jiang@cshs.org.

Abstract

Progressive tissue fibrosis is a cause of major morbidity and mortality. Pulmonary fibrosis is an epithelial-mesenchymal disorder in which TGF-β1 plays a central role in pathogenesis. Here we show that follistatin-like 1 (FSTL1) differentially regulates TGF-β and bone morphogenetic protein signaling, leading to epithelial injury and fibroblast activation. Haplodeletion of Fstl1 in mice or blockage of FSTL1 with a neutralizing antibody in mice reduced bleomycin-induced fibrosis in vivo. Fstl1 is induced in response to lung injury and promotes the accumulation of myofibroblasts and subsequent fibrosis. These data suggest that Fstl1 may serve as a novel therapeutic target for treatment of progressive lung fibrosis.

PMID:
25584011
PMCID:
PMC4322044
DOI:
10.1084/jem.20121878
[Indexed for MEDLINE]
Free PMC Article

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