《Journal of Experimental Medicine》 2:235-252 （2015）
Blocking follistatin-like 1 attenuates bleomycin-induced pulmonary fibrosis in mice
Dong, Y.; Geng, Y.; Li, L.; Li, X.; Yan, X.; Fang, Y.; Li, X.; Dong, S.; Liu, X.; Li, X.; Yang, X.; Zheng, X.; Xie, T.; Liang, J.; Dai, H.; Liu, X.; Yin, Z.; Noble, P. W.; Jiang, D.; Ning, W.
Progressive tissue fibrosis is a cause of major morbidity and mortality. Pulmonary fibrosis is an epithelial-mesenchymal disorder in which TGF-1 plays a central role in pathogenesis. Here we show that follistatin-like 1 (FSTL1) differentially regulates TGF- 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.