A biochemical link is proposed between recent observations on defective regulation of Cl− transport in CF respiratory epithelial cells and studies showing altered biological activity of calmodulin in exocrine glands from CF patients. A consensus is emerging that defective β-adrenergic secretory responsiveness in CF cells is caused by a defect in a regulator protein at a site distal to cyclic AMP formation. Our results indicate that this protein might be a specific calmodulin acceptor protein which modifies the activity of calmodulin in epithelial cells. Alteration in Ca2+/calmodulin dependent regulation of Cl− transport and protein secretion could explain (i) alterations in Ca2+ homeostasis seen in CF, (ii) defective β-adrenergic responses of CF cells, and (iii) the observed inability of cyclic AMP (acting via its specific protein kinase, A-kinase) to open apical membrane Cl− channels in CF epithelial cells. Most of the physiological abnormalities of CF including elevated sweat electrolytes and hyperviscous mucus can be explained on this basis.
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Review Article|
February 01 1988
Defective regulation of apical membrane chloride transport and exocytosis in cystic fibrosis
M. A. McPherson;
M. A. McPherson
1Department of Medical Biochemistry, University of Wales College of Medicine, Heath Park, Cardiff CF4 4XN, UK
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D. K. Shori;
D. K. Shori
1Department of Medical Biochemistry, University of Wales College of Medicine, Heath Park, Cardiff CF4 4XN, UK
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R. L. Dormer
R. L. Dormer
1Department of Medical Biochemistry, University of Wales College of Medicine, Heath Park, Cardiff CF4 4XN, UK
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Publisher: Portland Press Ltd
Received:
December 08 1987
Online ISSN: 1573-4935
Print ISSN: 0144-8463
© 1988 Plenum Publishing Corporation
1988
Biosci Rep (1988) 8 (1): 27–33.
Article history
Received:
December 08 1987
Citation
M. A. McPherson, D. K. Shori, R. L. Dormer; Defective regulation of apical membrane chloride transport and exocytosis in cystic fibrosis. Biosci Rep 1 February 1988; 8 (1): 27–33. doi: https://doi.org/10.1007/BF01128969
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