MassIVE MSV000085976

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Expression of a surfactant protein C mutation impairs postnatal expansion of alveolar type 2 epithelial cells leading to permanent cell loss and decreased repair capacity in adult lungs

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Description

Mutations in the gene encoding surfactant protein C (SFTPC) are associated with interstitial lung disease in children and adults. To assess the natural history of disease, we knocked-in a familial, disease-associated SFTPC mutation, L188Q (L184Q in mice), into the mouse Sftpc locus. Expression of the L184Q allele (LQ) resulted in formation of an unstable, misfolded proprotein (proSP-CLQ) that was rapidly degraded by the proteasome pathway. Translation of proSP-CLQ exceeded that of proSP-CWT in neonatal AT2 cells and was associated with transient activation of oxidative stress and apoptosis leading to impaired expansion of AT2 cells during postnatal alveolarization. Consequently, adult mutant mice demonstrated a decrease in the number of AT2 cells with no effect on lung homeostasis; however, lung regeneration in response to bleomycin challenge was substantially reduced in mutant mice. Collectively, these data support the hypothesis that susceptibility to disease in adult mutant mice is established during postnatal lung development and is associated with a permanent reduction in AT2 cell numbers. [doi:10.25345/C5D46W] [dataset license: CC0 1.0 Universal (CC0 1.0)]

Keywords: Surfactant protein C ; Alveolar type 2 epithelial cells

Contact

Principal Investigators:
(in alphabetical order) 		Timothy E. Weaver, Cincinnati Children's Hospital Medical Center, USA
Submitting User: snehasitaraman

Publications

Sitaraman S, Martin EP, Na CL, Zhao S, Green J, Deshmukh H, Perl AT, Bridges JP, Xu Y, Weaver TE.
Surfactant protein C mutation links postnatal type 2 cell dysfunction to adult disease.
JCI Insight. Epub 2021 Jun 17.

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