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Comment
. 2018 Aug 31;128(9):3745-3746.
doi: 10.1172/JCI122727. Epub 2018 Aug 13.

Lung injury and fibrosis induced by a mutant form of surfactant protein C

Timothy S Blackwell
Comment

Lung injury and fibrosis induced by a mutant form of surfactant protein C

Timothy S Blackwell. J Clin Invest. .

Abstract

Although mutant forms of the gene encoding surfactant protein C (SFTPC) have been linked to interstitial lung disease, the mechanisms by which the most common of these mutations, SFTPCI73T, results in lung fibrosis are uncertain. In this issue of the JCI, Nureki et al. developed a knockin mouse model and showed that SFTPCI73T is expressed by alveolar type II (AT2) epithelial cells in the lungs. These mice developed an age-related fibrotic phenotype when the mutant allele was expressed at low levels and acute lung inflammation/injury followed by lung fibrosis when mutant SFTPCI73T expression was enhanced. This work provides important information regarding the impact of AT2 cell dysfunction on fibrotic remodeling in the lungs.

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Conflict of interest statement

Conflict of interest: TSB declares research support from Boehringer Ingelheim and Celgene.

Comment on

References

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