PHENOTYPIC ABNORMALITIES IN LONG-TERM SURVIVING CYSTIC-FIBROSIS MICE

Citation
G. Kent et al., PHENOTYPIC ABNORMALITIES IN LONG-TERM SURVIVING CYSTIC-FIBROSIS MICE, Pediatric research, 40(2), 1996, pp. 233-241
Citations number
30
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Pediatrics
Journal title
ISSN journal
0031-3998
Volume
40
Issue
2
Year of publication
1996
Pages
233 - 241
Database
ISI
SICI code
0031-3998(1996)40:2<233:PAILSC>2.0.ZU;2-J
Abstract
Mouse models for cystic fibrosis (CF) with no CFTR function (Cftr(-/-) ) have the disadvantage that most animals die of intestinal obstructio n shortly after weaning. The objective of this research was to extend the lifespan of CF mice and characterize their phenotype. Weanlings we re placed on a nutrient liquid diet, and histologic and functional asp ects of organs implicated in the disease were subsequently examined. A pproximately 90% of Cftr(-/-) mice survived to 60 d, the majority beyo nd 100 d. Cftr(-/-) mice were underweight and had markedly abnormal in testinal histology. The intestinal epithelia did not respond to challe nges with agents that raised intracellular cAMP, consistent with the a bsence of functional CFTR. No lesions or functional abnormalities were evident in the lungs. Liquid-fed Cftr(-/-) mice were infertile, altho ugh some males weaned to a solid diet were fertile before they died. T hus, we have succeeded in using dietary means to prolong the lives of Cftr(-/-) mice.