Hydrophobic Surfactant Proteins in Lung Function and Disease
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- 26 December 2002
- journal article
- review article
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 347 (26) , 2141-2148
- https://doi.org/10.1056/nejmra022387
Abstract
Pulmonary surfactants consist of phospholipids and the hydrophobic proteins surfactant protein B and surfactant protein C. These surfactants keep the alveoli in the lung open to the atmosphere. Mutations in the genes encoding these proteins cause a variety of pulmonary syndromes. The pulmonary diseases associated with some of these mutations exemplify the consequences of the accumulation of misfolded proteins in tissue.Keywords
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