Ontology highlight
ABSTRACT:
SUBMITTER: Nureki SI
PROVIDER: S-EPMC6118576 | biostudies-literature | 2018 Aug
REPOSITORIES: biostudies-literature
Nureki Shin-Ichi SI Tomer Yaniv Y Venosa Alessandro A Katzen Jeremy J Russo Scott J SJ Jamil Sarita S Barrett Matthew M Nguyen Vivian V Kopp Meghan M Mulugeta Surafel S Beers Michael F MF
The Journal of clinical investigation 20180813 9
Epithelial cell dysfunction is postulated as an important component in the pathogenesis of idiopathic pulmonary fibrosis (IPF). Mutations in the surfactant protein C (SP-C) gene (SFTPC), an alveolar type II (AT2) cell-restricted protein, have been found in sporadic and familial IPF. To causally link these events, we developed a knockin mouse model capable of regulated expression of an IPF-associated isoleucine-to-threonine substitution at codon 73 (I73T) in Sftpc (SP-CI73T). Tamoxifen-treated SP ...[more]