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: 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 been linked to clinical IPF. To better understand the sequence of events that impact AE-IPF, we leveraged a murine model of inducible SP-C^I73T (SP-C^I73T/I73TFlp^+/- ) expression. Following administration of tamoxifen to 8-12-wk-old mice, an upregulation of Sftpc^I73T initiated a diffuse lung injury marked by increases in bronchoalv

SUBMITTER: Venosa A 

PROVIDER: S-EPMC6478557 | biostudies-literature | 2019 May

REPOSITORIES: biostudies-literature