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

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Glasser, S. W. Ikegami, M. Korfhagen, T. R. Ross, G. F. Whitsett, J. A. Na, C. L. Sly, P. D. Burhans, M. S.
Description	Author Affiliation: Glasser SW ( Division of Pulmonary Biology, Children's Hospital Medical Center, Cincinnati, OH 45229-3039, USA. glass0@chmcc.org);
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.
ISSN	00278424
e-ISSN	10916490
Journal	Proceedings of the National Academy of Sciences of the United States of America
Issue Number	11
Volume Number	98
Language	English
Publisher	National Academy of Sciences
Publisher Date	2001-05-01
Publisher Place	United States
Access Restriction	Open
Subject Keyword	Lung Physiology Proteolipids Pulmonary Surfactants Animals Bronchoalveolar Lavage Glycoproteins Metabolism Pathology Lung Volume Measurements Mice Mice, Inbred C57BL Mice, Knockout Phosphatidylcholines Genetics Pulmonary Surfactant-Associated Protein D Pulmonary Surfactant-Associated Proteins Research Support, U.S. Gov't, P.H.S. Multidisciplinary
Content Type	Text
Resource Type	Article
Subject	Multidisciplinary

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