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

Am J Physiol. 1988 May;254(5 Pt 1):C719-24. doi: 10.1152/ajpcell.1988.254.5.C719.


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.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Amiloride / pharmacology
  • Biological Transport, Active
  • Carrier Proteins / metabolism*
  • Chlorides / metabolism
  • Cyclic AMP Receptor Protein*
  • Cystic Fibrosis / metabolism*
  • Female
  • Humans
  • Ions / metabolism*
  • Male
  • Molecular Weight
  • Permeability
  • Rectum / metabolism*
  • Theophylline / pharmacology


  • Carrier Proteins
  • Chlorides
  • Cyclic AMP Receptor Protein
  • Ions
  • Amiloride
  • Theophylline