Defective regulation of epithelial Cl- permeability and protein secretion in cystic fibrosis: the putative basic defect.

American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation
K L Kirk

Abstract

The search for a basic functional defect in CF appears to be converging on a defect in the regulation of epithelial Cl- permeability and perhaps protein secretion. Fundamental issues that remain unresolved include (1) the identity of the CF gene, (2) the precise role played by the CF gene product in regulating Cl- permeability and protein secretion, and (3) the identities and properties of alternate pathways for regulating Cl- permeability and protein secretion that are not compromised in CF. The first issue should be resolved in the near future as molecular genetic approaches are used to pinpoint the location of the CF locus on chromosome 7. The second issue is more complex and will require the development of generally useful assays of Cl- permeability and protein secretion that can be used to assess the abilities of candidate CF gene products to complement, or correct, the functional defect in CF cells. Characterizing the precise function of the CF gene product may be difficult if the regulatory pathways that control these cellular processes are complex (ie, involve multiple regulatory steps and second messengers) or if the CF gene is a regulatory gene (rather than a structural gene) that represses or induces the synthesis of...Continue Reading

References

Jun 15, 1988·Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences·L C TsuiM Buchwald
Jun 1, 1984·The Journal of Clinical Investigation·K Sato, F Sato
Feb 3, 1983·Nature·P M Quinton

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Citations

Feb 26, 1998·Current Problems in Diagnostic Radiology·T B Hunter, N Carlevato

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