Altered stability of pulmonary surfactant in SP-C-deficient mice

Glasser, Stephan W., Burhans, Michael S., Korfhagen, Thomas R., Na, Cheng-Lun, Sly, Peter D., Ross, Gary F., Ikegami, Machiko and Whitsett, Jeffrey A. (2001) Altered stability of pulmonary surfactant in SP-C-deficient mice. Proceedings of the National Academy of Sciences of the United States of America, 98 11: 6366-6371. doi:10.1073/pnas.101500298

Author Glasser, Stephan W.
Burhans, Michael S.
Korfhagen, Thomas R.
Na, Cheng-Lun
Sly, Peter D.
Ross, Gary F.
Ikegami, Machiko
Whitsett, Jeffrey A.
Title Altered stability of pulmonary surfactant in SP-C-deficient mice
Journal name Proceedings of the National Academy of Sciences of the United States of America   Check publisher's open access policy
ISSN 0027-8424
Publication date 2001-05-01
Year available 2001
Sub-type Article (original research)
DOI 10.1073/pnas.101500298
Open Access Status Not Open Access
Volume 98
Issue 11
Start page 6366
End page 6371
Total pages 6
Place of publication Washington, DC, United States
Publisher National Academy of Sciences
Language eng
Abstract The surfactant protein C (SP-C) gene encodes an extremely hydrophobic, 4-kDa peptide produced by alveolar epithelial cells in the lung. To discern the role of SP-C in lung function, SP-C-deficient (-/-) mice were produced. The SP-C (-/-) mice were viable at birth and grew normally to adulthood without apparent pulmonary abnormalities. SP-C mRNA was not detected in the lungs of SP-C (-/-) mice, nor was mature SP-C protein detected by Western blot of alveolar lavage from SP-C (-/-) mice. The levels of the other surfactant proteins (A, B, D) in alveolar lavage were comparable to those in wild-type mice. Surfactant pool sizes, surfactant synthesis, and lung morphology were similar in SP-C (-/-) and SP-C (+/+) mice. Lamellar bodies were present in SP-C (-/-) type II cells, and tubular myelin was present in the alveolar lumen. Lung mechanics studies demonstrated abnormalities in lung hysteresivity (a term used to reflect the mechanical coupling between energy dissipative forces and tissue-elastic properties) at low, positive-end, expiratory pressures. The stability of captive bubbles with surfactant from the SP-C (-/-) mice was decreased significantly, indicating that Sp-C plays a role in the stabilization of surfactant at low lung volumes, a condition that may accompany respiratory distress syndrome in infants and adults.
Keyword Gene targeted mice
Acute lung injury
Protein C
Respiratory epithelium
Q-Index Code C1
Q-Index Status Provisional Code
Grant ID HL38865
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: School of Medicine Publications
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Created: Wed, 17 Nov 2010, 21:27:23 EST