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The interstitial lung diseases (ILD) are a heterogeneous group of pathologies that include the lethal fibrotic disease idiopathic pulmonary fibrosis (IPF). IPF is a chronic, progressive fibrotic interstitial pneumonia of unknown cause with a median survival of approximately three years. There is no treatment for IPF and no diagnostic procedure to identify at-risk individuals or predict disease progression in asymptomatic patients; further, none of the current animal models mimics the entire disease. For these reasons, generation of an appropriate mouse model of IPF is the central focus of our current research activity.
Mutations in the SFTPC gene are associated with development of interstitial lung disease (ILD) in both children and adults. The SFTPC gene is expressed exclusively in type II epithelial cells, implicating this cell type in the pathogenesis of ILD. We are currently using transgenic technology to “knockin” disease-associated alleles into the mouse Sftpc locus. These mice will genocopy human patients with ILD / IPF and facilitate characterization of the natural history of the disease, identification of biomarkers for diagnosis and prediction of disease progression, and development of new treatment strategies.
Bridges JP, Xu Y, Na CL, Wong HR, Weaver TE. Adaptation and increased susceptibility to infection associated with constitutive expression of misfolded SP-C. J. Cell Biol. 172:395-407. 2006.
Bridges JP, Wert SE, Nogee LM, Weaver TE. Expression of a human surfactant protein C mutation associated with interstitial lung disease disrupts lung development in transgenic mice. J. Biol. Chem. 278:52739-52746. 2003.
Conkright JJ, Na CL, Weaver TE. Overexpression of surfactant protein-C mature peptide causes neonatal lethality in transgenic mice. Am J Respir Cell Mol Biol. 26:85-90. 2002.
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Histological sections of normal lung tissue (left panel) and lung tissue from a child with interstitial lung disease (right panel). Note the thickness of the air-blood barrier (tissue between blue arrows).
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