|  Help  |  About  |  Contact Us

Publication : Cystic fibrosis growth retardation is not correlated with loss of Cftr in the intestinal epithelium.

First Author  Hodges CA Year  2011
Journal  Am J Physiol Gastrointest Liver Physiol Volume  301
Issue  3 Pages  G528-36
PubMed ID  21659619 Mgi Jnum  J:234604
Mgi Id  MGI:5790312 Doi  10.1152/ajpgi.00052.2011
Citation  Hodges CA, et al. (2011) Cystic fibrosis growth retardation is not correlated with loss of Cftr in the intestinal epithelium. Am J Physiol Gastrointest Liver Physiol 301(3):G528-36
abstractText  Maldigestion due to exocrine pancreatic insufficiency leads to intestinal malabsorption and consequent malnutrition, a mechanism proposed to cause growth retardation associated with cystic fibrosis (CF). However, although enzyme replacement therapy combined with increased caloric intake improves weight gain, the effect on stature is not significant, suggesting that growth retardation has a more complex etiology. Mouse models of CF support this, since these animals do not experience exocrine pancreatic insufficiency yet are growth impaired. Cftr absence from the intestinal epithelium has been suggested as a primary source of growth retardation in CF mice, a concept we directly tested by generating mouse models with Cftr selectively inactivated or restored in intestinal epithelium. The relationship between growth and functional characteristics of the intestines, including transepithelial electrophysiology, incidence of intestinal obstruction, and histopathology, were assessed. Absence of Cftr exclusively from intestinal epithelium resulted in loss of cAMP-stimulated short-circuit current, goblet cell hyperplasia, and occurrence of intestinal obstructions but only slight and transient impaired growth. In contrast, specifically restoring Cftr to the intestinal epithelium resulted in restoration of ion transport and completely protected against obstruction and histopathological anomalies, but growth was indistinguishable from CF mice. These results indicate that absence of Cftr in the intestinal epithelium is an important contributor to the intestinal obstruction phenotype in CF but does not correlate with the observed growth reduction in CF.
Quick Links:
 
Quick Links:
 

Expression

Publication --> Expression annotations

 

Other

10 Bio Entities

0 Expression