| First Author | Geiser M | Year | 2003 |
| Journal | Am J Physiol Lung Cell Mol Physiol | Volume | 285 |
| Issue | 6 | Pages | L1277-85 |
| PubMed ID | 12936909 | Mgi Jnum | J:87133 |
| Mgi Id | MGI:2683782 | Doi | 10.1152/ajplung.00128.2003 |
| Citation | Geiser M, et al. (2003) Surface-lining layer of airways in cystic fibrosis mice. Am J Physiol Lung Cell Mol Physiol 285(6):L1277-85 |
| abstractText | Lung disease is the major cause of death in individuals suffering from cystic fibrosis (CF), with abnormal lung-lining fluids occurring as early as early infancy. However, the precise etiology of CF lung disease is still poorly understood. We investigated the structural components of the airway surface-lining layer in targeted Cftrtm1HGU/Cftrtm1HGU mutant mice and non-CF controls. Five lungs per animal group were fixed by intravascular triple perfusion. The ultrastructure of the surface-lining layer of large and small intrapulmonary conducting airways was systematically investigated according to a standard protocol in transmission and scanning electron micrographs. In both animal groups, the surface-lining layer consisted of an aqueous phase and an osmiophilic film of variable thickness at the air-fluid interface. The aqueous phase usually did extend <1 microm beyond the uppermost tips of the epithelial cells in both animal groups. The aqueous phase of the small airways was slightly more electron dense in Cftrtm1HGU/Cftrtm1HGU than in non-CF mice. Neither the ultrastructure of the surfactant film at the air-fluid interface nor the forms assumed by the osmiophilic structures associated with surfactant turnover in the aqueous layer differed significantly in Cftrtm1HGU/Cftrtm1HGU and non-CF mice. Hence, there were no signs of any ultrastructural abnormalities in the surface-lining layer of young adult Cftrtm1HGU/Cftrtm1HGU mice before infection with CF-related pathogens. |
