Outwardly rectifying chloride channels and CF: a divorce and remarriage
- PMID: 7680029
- DOI: 10.1007/BF00768065
Outwardly rectifying chloride channels and CF: a divorce and remarriage
Abstract
Outwardly rectifying Cl- channels were originally thought to be the central element in cystic fibrosis. The role of these channels in CF was questioned to such an extent that doubts were raised about the validity of the original experiments. Recent data reestablishes a role for outwardly rectifying Cl- channels (ORCC) in CF and suggests that the protein encoded by the CF gene, the cystic fibrosis transmembrane regulator (CFTR), can effect the regulation of more than one channel in the airway. This minireview deals with the rise, fall, and resurrection of the role of outwardly rectifying Cl- channels in CF.
Similar articles
-
Defective regulation of outwardly rectifying Cl- channels by protein kinase A corrected by insertion of CFTR.Nature. 1992 Aug 13;358(6387):581-4. doi: 10.1038/358581a0. Nature. 1992. PMID: 1380129
-
CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship.Nature. 1993 May 20;363(6426):263-8. doi: 10.1038/363263a0. Nature. 1993. PMID: 7683773
-
The basic defect in cystic fibrosis.Trends Biochem Sci. 1991 Dec;16(12):474-7. doi: 10.1016/0968-0004(91)90183-v. Trends Biochem Sci. 1991. PMID: 1723552 Review.
-
Cystic fibrosis, the CFTR, and rectifying Cl- channels.Adv Exp Med Biol. 1991;290:253-69; discussion 269-72. doi: 10.1007/978-1-4684-5934-0_25. Adv Exp Med Biol. 1991. PMID: 1719763 Review.
-
Both CFTR and outwardly rectifying chloride channels contribute to cAMP-stimulated whole cell chloride currents.Am J Physiol. 1994 May;266(5 Pt 1):C1464-77. doi: 10.1152/ajpcell.1994.266.5.C1464. Am J Physiol. 1994. PMID: 7515570
Cited by
-
Cystic fibrosis transmembrane conductance regulator-associated ATP release is controlled by a chloride sensor.J Cell Biol. 1998 Nov 2;143(3):645-57. doi: 10.1083/jcb.143.3.645. J Cell Biol. 1998. PMID: 9813087 Free PMC article.
-
Gene therapy for cystic fibrosis.Clin Rev Allergy Immunol. 2008 Dec;35(3):164-78. doi: 10.1007/s12016-008-8080-3. Clin Rev Allergy Immunol. 2008. PMID: 18600482 Review.
-
Bicarbonate conductance and pH regulatory capability of cystic fibrosis transmembrane conductance regulator.Proc Natl Acad Sci U S A. 1994 Jun 7;91(12):5340-4. doi: 10.1073/pnas.91.12.5340. Proc Natl Acad Sci U S A. 1994. PMID: 7515498 Free PMC article.
-
Mechanisms of lipid malabsorption in Cystic Fibrosis: the impact of essential fatty acids deficiency.Nutr Metab (Lond). 2005 May 3;2(1):11. doi: 10.1186/1743-7075-2-11. Nutr Metab (Lond). 2005. PMID: 15869703 Free PMC article.
References
Publication types
MeSH terms
Substances
Grants and funding
LinkOut - more resources
Medical