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Rectum has abnormal ion transport but normal cAMP-binding proteins in cystic fibrosis

Rectum has abnormal ion transport but normal cAMP-binding proteins in cystic fibrosis L., NANCY is normal in airways and sweat glands (6, 27, 30). Research efts in have been hampered by the lack of easily available and adequate tissue in vivo and in vitro studies. The is an ion-transporting epithelium that is easily accessible and is relatively unaffected by secondary pathological changes in (13). Although several studies (9,16,21,22,31) have compared basal- and amiloride-inhibitable transrectal potential difference (PD) in control and subjects, there has been variability in the results and none of these studies have examined Cl permeability or CAMP responsiveness. In the present study, we examined whether the of subjects expresses abnormalities in Cl permeability in vivo and whether we can attribute these changes to an impairment in CAMP generation and/or CAMP-specific binding proteins. Our findings indicate that in the exhibits abnormalities in Cl permeability and in theophylline responsiveness, but has normal CAMP generation and CAMP-binding proteins. MATERIAL AND METHODS In Vivo Rectal PD Measurements Subject selection and preparation. Six (5 male and 1 female) subjects with a documented abnormal sweat Cl test and above age 18 yr (27.7 t 0.7 yr; mean -+ SE) participated after inmed consent was obtained. These studies were approved by the Institutional Review Board. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png AJP - Cell Physiology The American Physiological Society

Rectum has abnormal ion transport but normal cAMP-binding proteins in cystic fibrosis

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Publisher
The American Physiological Society
Copyright
Copyright © 1988 the American Physiological Society
ISSN
0363-6143
eISSN
1522-1563
Publisher site
See Article on Publisher Site

Abstract

L., NANCY is normal in airways and sweat glands (6, 27, 30). Research efts in have been hampered by the lack of easily available and adequate tissue in vivo and in vitro studies. The is an ion-transporting epithelium that is easily accessible and is relatively unaffected by secondary pathological changes in (13). Although several studies (9,16,21,22,31) have compared basal- and amiloride-inhibitable transrectal potential difference (PD) in control and subjects, there has been variability in the results and none of these studies have examined Cl permeability or CAMP responsiveness. In the present study, we examined whether the of subjects expresses abnormalities in Cl permeability in vivo and whether we can attribute these changes to an impairment in CAMP generation and/or CAMP-specific binding proteins. Our findings indicate that in the exhibits abnormalities in Cl permeability and in theophylline responsiveness, but has normal CAMP generation and CAMP-binding proteins. MATERIAL AND METHODS In Vivo Rectal PD Measurements Subject selection and preparation. Six (5 male and 1 female) subjects with a documented abnormal sweat Cl test and above age 18 yr (27.7 t 0.7 yr; mean -+ SE) participated after inmed consent was obtained. These studies were approved by the Institutional Review Board.

Journal

AJP - Cell PhysiologyThe American Physiological Society

Published: May 1, 1988

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