Division of Allergy, Pulmonary and Critical Care Medicine, Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA. Department of Veterans Affairs Medical Center, Nashville, Tennessee, USA. Department of Cell and Developmental Biology, Vanderbilt University School of Medicine, Nashville, Tennessee, USA.
Address correspondence to: Timothy S. Blackwell, Division of Allergy, Pulmonary and Critical Care Medicine, Vanderbilt University School of Medicine, 1611 21st Avenue South, T-1218 Medical Center North, Nashville, Tennessee 37232, USA. Phone: 615.322.3412; Email: email@example.com.
First published August 13, 2018 - More info
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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