Epithelial Expression of an Interstitial Lung Disease–Associated Mutation in Surfactant Protein-C Modulates Recruitment and Activation of Key Myeloid Cell Populations in Mice

Abstract excerpt

Abstract Patients with idiopathic pulmonary fibrosis (IPF) often experience precipitous deteriorations, termed “acute exacerbations” (AE), marked by diffuse alveolitis and altered gas exchange, resulting in a significant loss of lung function or mortality. The missense isoleucine to threonine substitution at position 73 (I73T) in the alveolar type 2 cell-restricted surfactant protein-C (SP-C) gene (SFTPC) has...

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