Extract
Alveolar lining fluid requires surfactant to prevent alveolar collapse at end expiration. This activity is generated by interactions of phospholipids with the hydrophobic surfactant proteins SP-B and -C [1]. Mutations in SP-B and -C cause familial pulmonary fibrosis [2, 3]. Whilst loss of surfactant activity is associated with respiratory distress syndromes, this does not seem to be the main driver of disease caused by SP-C mutations [4, 5], indicating there is instead a pathogenic gain-of-function. What is going on, and why should we care?
Abstract
Dysfunction in protein folding and intracellular processing are important mediators of disease. Detailed characterisation of SP-C deficiency mutations illuminates a pathway to alveolar epithelial cell damage that triggers familial pulmonary fibrosis. https://bit.ly/3KavSgu
Footnotes
Conflict of interest: B. Gooptu reports grant funding from MRC (UK), British Lung Foundation, Alpha-1 Foundation and NIHR; consultancy fees from Vertex; and support in kind for collaborative research into pulmonary fibrosis from Galecto Biotech.
Support statement: This work was supported by NIHR Leicester Biomedical Research Centre, Alpha-1 Foundation, Medical Research Council and British Lung Foundation. Funding information for this article has been deposited with the Crossref Funder Registry.
- Received August 5, 2021.
- Accepted December 27, 2021.
- Copyright ©The authors 2022. For reproduction rights and permissions contact permissions{at}ersnet.org
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