Article
Adaptation and increased susceptibility to infection associated with constitutive expression of misfolded SP-C.
The Journal of cell biology - 30 Jan 2006
Bridges James P, Xu Yan, Na Cheng-Lun, Wong Hector R, Weaver Timothy E
Abstract excerpt
Mutations in the gene encoding SP-C (surfactant protein C; SFTPC) have been linked to interstitial lung disease (ILD) in children and adults. Expression of the index mutation, SP-C(Deltaexon4), in transiently transfected cells and type II cells of transgenic mice resulted in misfolding of the proprotein, activation of endoplasmic reticulum (ER) stress pathways, and cytotoxicity. In this study, we show that stably...
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