Kunzelmann, Karl and Ousingsawat, Jiraporn and Kraus, Andre and Park, Julien H. and Marquardt, Thorsten and Schreiber, Rainer and Buchholz, Björn (2023) Pathogenic Relationships in Cystic Fibrosis and Renal Diseases: CFTR, SLC26A9 and Anoctamins. INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES, 24 (17): 13278. ISSN 1661-6596, 1422-0067
Full text not available from this repository. (Request a copy)Abstract
The Cl--transporting proteins CFTR, SLC26A9, and anoctamin (ANO1; ANO6) appear to have more in common than initially suspected, as they all participate in the pathogenic process and clinical outcomes of airway and renal diseases. In the present review, we will therefore concentrate on recent findings concerning electrolyte transport in the airways and kidneys, and the role of CFTR, SLC26A9, and the anoctamins ANO1 and ANO6. Special emphasis will be placed on cystic fibrosis and asthma, as well as renal alkalosis and polycystic kidney disease. In essence, we will summarize recent evidence indicating that CFTR is the only relevant secretory Cl- channel in airways under basal (nonstimulated) conditions and after stimulation by secretagogues. Information is provided on the expressions of ANO1 and ANO6, which are important for the correct expression and function of CFTR. In addition, there is evidence that the Cl- transporter SLC26A9 expressed in the airways may have a reabsorptive rather than a Cl--secretory function. In the renal collecting ducts, bicarbonate secretion occurs through a synergistic action of CFTR and the Cl-/HCO3- transporter SLC26A4 (pendrin), which is probably supported by ANO1. Finally, in autosomal dominant polycystic kidney disease (ADPKD), the secretory function of CFTR in renal cyst formation may have been overestimated, whereas ANO1 and ANO6 have now been shown to be crucial in ADPKD and therefore represent new pharmacological targets for the treatment of polycystic kidney disease.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | TRANSMEMBRANE CONDUCTANCE REGULATOR; PROTEIN-KINASE-C; ACTIVATED CHLORIDE CHANNELS; EPITHELIAL-CELLS; KIDNEY-DISEASE; ION-TRANSPORT; DEPENDENT ACTIVATION; OXIDATIVE STRESS; K+ CHANNELS; R-DOMAIN; TMEM16A; TMEM16F; anoctamin; CFTR; pendrin |
| Subjects: | 500 Science > 570 Life sciences 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Biology, Preclinical Medicine > Institut für Physiologie > Prof. Dr. Karl Kunzelmann |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 19 Mar 2024 10:07 |
| Last Modified: | 19 Mar 2024 10:07 |
| URI: | https://pred.uni-regensburg.de/id/eprint/59661 |
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