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Blocking Follistatin-like 1 attenuates pulmonary fibrosis in mice

Yan Dong, Wen Ning

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Abstract

Idiopathic pulmonary fibrosis (IPF) is an incurable fibrosing disorder that progresses relentlessly to respiratory failure.Follistatin-like 1 (Fstl1) is a TGF-b1 induced secretory glycoprotein whose functions are largely unknown.Here we showed that Fstl1 was up-regulated in lung tissue and lung primary fibroblasts from IPF patients,as well as in bleomycin-induced lung fibrosis in mice.Fstl1+/-mice were resistant to bleomycin-induced lung injury and displayed an attenuated interstitial fibrotic phenotype.

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What this paper is about

Idiopathic pulmonary fibrosis (IPF) is an incurable fibrosing disorder that progresses relentlessly to respiratory failure.Follistatin-like 1 (Fstl1) is a TGF-b1 induced secretory glycoprotein whose functions are largely unknown.Here we showed that Fstl1 was up-regulated in lung tissue and lung primary fibroblasts from IPF patients,as well as in bleomycin-induced lung fibrosis in mice.Fstl1+/-mice were resistant to bleomycin-induced lung injury and displayed an attenuated interstitial fibrotic phenotype.

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Available abstract

Idiopathic pulmonary fibrosis (IPF) is an incurable fibrosing disorder that progresses relentlessly to respiratory failure.Follistatin-like 1 (Fstl1) is a TGF-b1 induced secretory glycoprotein whose functions are largely unknown.Here we showed that Fstl1 was up-regulated in lung tissue and lung primary fibroblasts from IPF patients,as well as in bleomycin-induced lung fibrosis in mice.Fstl1+/-mice were resistant to bleomycin-induced lung injury and displayed an attenuated interstitial fibrotic phenotype.

Key concepts: Bleomycin, Follistatin, Idiopathic pulmonary fibrosis, Pulmonary fibrosis, Lung, Medicine, Fibrosis, Lung fibrosis

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