Document Detail

Rectum has abnormal ion transport but normal cAMP-binding proteins in cystic fibrosis.
MedLine Citation:
PMID:  3364557     Owner:  NLM     Status:  MEDLINE    
The luminal membranes of involved tissues in cystic fibrosis (CF) are relatively impermeable to Cl and the regulation of Cl transport by adenosine 3',5'-cyclic monophosphate (cAMP)-mediated hormones is abnormal. We investigated the human rectum as a putative model for CF. We compared in vivo transrectal potential difference (PD) in CF and in normal subjects in response to sequential perfusions with various test solutions. The base-line PD was different in normal (-35.5 +/- 4.0 mV; lumen negative; mean +/- SE; n = 9) and CF subjects (-23.4 +/- 3.1 mV; n = 6; P less than 0.025) and was eliminated by amiloride (10(-4) M) perfusion in both groups by 3 min. However, in response to a Cl-free solution with amiloride, all six CF subjects exhibit less of a change in PD (PD, -2.2 +/- 1.2 mV vs. -11.7 +/- 1.5 mV in 6 controls; P less than 0.01). Furthermore, normal subjects (n = 7) respond to a 5 mM theophylline + amiloride perfusion with an increase in lumen-negative PD, whereas, CF subjects (n = 6) show no increase in lumen-negative PD. Rectal biopsy specimens from four normal and four CF subjects exhibit similar (2- to 3-fold) increases in theophylline-induced cAMP content and have similar cAMP-binding proteins (CF, n = 3; control, n = 3). We conclude that the rectum is an involved epithelium in CF in which the aberration may lie at a point beyond the binding of cAMP to its protein kinase.
J L Goldstein; N T Nash; F al-Bazzaz; T J Layden; M C Rao
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Publication Detail:
Type:  Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  The American journal of physiology     Volume:  254     ISSN:  0002-9513     ISO Abbreviation:  Am. J. Physiol.     Publication Date:  1988 May 
Date Detail:
Created Date:  1988-06-09     Completed Date:  1988-06-09     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  0370511     Medline TA:  Am J Physiol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  C719-24     Citation Subset:  IM    
Department of Medicine, University of Illinois College of Medicine, Chicago.
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MeSH Terms
Amiloride / pharmacology
Biological Transport, Active
Carrier Proteins / metabolism*
Chlorides / metabolism
Cyclic AMP Receptor Protein*
Cystic Fibrosis / metabolism*
Ions / metabolism*
Molecular Weight
Rectum / metabolism*
Theophylline / pharmacology
Grant Support
Reg. No./Substance:
0/Carrier Proteins; 0/Chlorides; 0/Cyclic AMP Receptor Protein; 0/Ions; 2609-46-3/Amiloride; 58-55-9/Theophylline

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