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CFTR  -  cystic fibrosis transmembrane conductance...

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Disease relevance of CFTR

 

High impact information on CFTR

  • The presence of CFTR in neurons localized to these regions of brain controlling homeostasis and energy expenditure may elucidate the pathogenesis of other nonpulmonary and gastrointestinal manifestations which commonly are observed in children with cystic fibrosis [5].
  • The expression of CFTR mRNA and protein in discrete areas of brain, including the hypothalamus, thalamus, and amygdaloid nuclei, which are involved in regulation of appetite and resting energy expenditure, is identical [5].
  • In previous studies we have characterized the expression of the cystic fibrosis transmembrane conductance regulator (CFTR) protein in clathrin-coated vesicles derived from bovine brain and in neurons of rat brain [5].
  • Protein kinase C (PKC) phosphorylation stimulates the cystic fibrosis transmembrane conductance regulator (CFTR) channel and enhances its activation by protein kinase A (PKA) through mechanisms that remain poorly understood [6].
  • These results identify functionally important PKC consensus sequences on CFTR and will facilitate studies of its convergent regulation by PKC and PKA [6].
 

Chemical compound and disease context of CFTR

 

Biological context of CFTR

 

Anatomical context of CFTR

  • In epithelial cells, which do not express CFTR, trans-Golgi pH was (in 25 mM HCO3-) 6.36 +/- 0.04 (n = 33) and 6.34 +/- 0.08 (n = 23, CPT-cAMP) in MDCK cells and 6.25 +/- 0.04 (n = 18) and 6.24 +/- 0.06 (n = 15, CPT-cAMP) in SK-MES-1 cells [12].
  • After stimulation of plasma membrane Cl- conductance by 8-(4-chlorophenylthio)-cAMP (CPT-cAMP), trans-Golgi pH was 6.42 +/- 0.07 (n = 22, control), 6.47 +/- 0.07 (n = 20, CFTR), and 6.35 +/- 0 [12].
  • PTX treatment of a CFTR-immunodepleted protein preparation incorporated into bilayer membranes resulted in a similar increase in the Po of the larger conductance channel and restored PKA-sensitivity that was lost after CFTR immunodepletion [13].
  • In unstimulated fibroblasts in HCO3--free buffer, trans- Golgi pH was 6.25 +/- 0.04 (mean +/- S.E.; n = 80, vector control), 6.30 +/- 0.03 (n = 74, CFTR) and 6.23 +/- 0.06 (n = 60, DeltaF508) (not significant) [12].
  • To test whether CFTR expression affects pH in the endosomal compartment in HCO3- buffer, pH was measured by ratio imaging in individual endosomes labeled with fluorescein-rhodamine dextrans [12].
 

Associations of CFTR with chemical compounds

  • In contrast, the P-glycoprotein inhibitors tamoxifen and verapamil and the cystic fibrosis transmembrane conductance regulator (CFTR) blockers glybenclamide and diphenylamine-2-carboxylate did not affect ATP release from either cell type [14].
  • Similarly, significant pH differences were not found for control versus CFTR-expressing cells in 25 mM HCO3- buffer [12].
  • Moreover, the addition of hexokinase + glucose to the extracellular side prevented activation of the ORCCs by PKA and ATP in the presence of CFTR [15].
  • We now report the purification of CFTR from bovine tracheal epithelia and the purification of a CFTR conduction mutant (G551D CFTR) from retrovirally transduced mouse L cells using a combination of alkali stripping, Triton-X extraction, and immunoaffinity chromatography [15].
  • In summary, we conclude that CFTR is present in the apical membrane of bovine corneal endothelium and could contribute to transendothelial Cl(-) and HCO transport [16].
 

Physical interactions of CFTR

 

Analytical, diagnostic and therapeutic context of CFTR

References

  1. Noninvasive in vivo fluorescence measurement of airway-surface liquid depth, salt concentration, and pH. Jayaraman, S., Song, Y., Vetrivel, L., Shankar, L., Verkman, A.S. J. Clin. Invest. (2001) [Pubmed]
  2. Cystic fibrosis transmembrane conductance regulator protein expression in brain. Mulberg, A.E., Wiedner, E.B., Bao, X., Marshall, J., Jefferson, D.M., Altschuler, S.M. Neuroreport (1994) [Pubmed]
  3. Transfected beta3- but not beta2-adrenergic receptors regulate cystic fibrosis transmembrane conductance regulator activity via a new pathway involving the mitogen-activated protein kinases extracellular signal-regulated kinases. Robay, A., Toumaniantz, G., Leblais, V., Gauthier, C. Mol. Pharmacol. (2005) [Pubmed]
  4. Origin and utility of the reverse dot-blot. Gold, B. Expert Rev. Mol. Diagn. (2003) [Pubmed]
  5. Expression and localization of the cystic fibrosis transmembrane conductance regulator mRNA and its protein in rat brain. Mulberg, A.E., Resta, L.P., Wiedner, E.B., Altschuler, S.M., Jefferson, D.M., Broussard, D.L. J. Clin. Invest. (1995) [Pubmed]
  6. Stimulatory and inhibitory protein kinase C consensus sequences regulate the cystic fibrosis transmembrane conductance regulator. Chappe, V., Hinkson, D.A., Howell, L.D., Evagelidis, A., Liao, J., Chang, X.B., Riordan, J.R., Hanrahan, J.W. Proc. Natl. Acad. Sci. U.S.A. (2004) [Pubmed]
  7. Cell volume response to hyposmotic shock and elevated cAMP in bovine trabecular meshwork cells. Srinivas, S.P., Maertens, C., Goon, L.H., Goon, L., Satpathy, M., Yue, B.Y., Droogmans, G., Nilius, B. Exp. Eye Res. (2004) [Pubmed]
  8. Phosphorylation of protein kinase C sites in NBD1 and the R domain control CFTR channel activation by PKA. Chappe, V., Hinkson, D.A., Zhu, T., Chang, X.B., Riordan, J.R., Hanrahan, J.W. J. Physiol. (Lond.) (2003) [Pubmed]
  9. A comparative genomic analysis of the cow, pig, and human CFTR genes identifies potential intronic regulatory elements. Williams, S.H., Mouchel, N., Harris, A. Genomics (2003) [Pubmed]
  10. A cross-species analysis of the cystic fibrosis transmembrane conductance regulator. Potential functional domains and regulatory sites. Diamond, G., Scanlin, T.F., Zasloff, M.A., Bevins, C.L. J. Biol. Chem. (1991) [Pubmed]
  11. Cross-species characterization of the promoter region of the cystic fibrosis transmembrane conductance regulator gene reveals multiple levels of regulation. Vuillaumier, S., Dixmeras, I., Messaï, H., Lapouméroulie, C., Lallemand, D., Gekas, J., Chehab, F.F., Perret, C., Elion, J., Denamur, E. Biochem. J. (1997) [Pubmed]
  12. Evidence against defective trans-Golgi acidification in cystic fibrosis. Seksek, O., Biwersi, J., Verkman, A.S. J. Biol. Chem. (1996) [Pubmed]
  13. G-protein regulation of outwardly rectified epithelial chloride channels incorporated into planar bilayer membranes. Ismailov, I.I., Jovov, B., Fuller, C.M., Berdiev, B.K., Keeton, D.A., Benos, D.J. J. Biol. Chem. (1996) [Pubmed]
  14. A release mechanism for stored ATP in ocular ciliary epithelial cells. Mitchell, C.H., Carré, D.A., McGlinn, A.M., Stone, R.A., Civan, M.M. Proc. Natl. Acad. Sci. U.S.A. (1998) [Pubmed]
  15. Interaction between cystic fibrosis transmembrane conductance regulator and outwardly rectified chloride channels. Jovov, B., Ismailov, I.I., Berdiev, B.K., Fuller, C.M., Sorscher, E.J., Dedman, J.R., Kaetzel, M.A., Benos, D.J. J. Biol. Chem. (1995) [Pubmed]
  16. Expression, localization, and functional evaluation of CFTR in bovine corneal endothelial cells. Sun, X.C., Bonanno, J.A. Am. J. Physiol., Cell Physiol. (2002) [Pubmed]
  17. The molecular chaperone Hsc70 assists the in vitro folding of the N-terminal nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator. Strickland, E., Qu, B.H., Millen, L., Thomas, P.J. J. Biol. Chem. (1997) [Pubmed]
  18. Cystic fibrosis transmembrane conductance regulator is required for protein kinase A activation of an outwardly rectified anion channel purified from bovine tracheal epithelia. Jovov, B., Ismailov, I.I., Benos, D.J. J. Biol. Chem. (1995) [Pubmed]
  19. Airway surface liquid osmolality measured using fluorophore-encapsulated liposomes. Jayaraman, S., Song, Y., Verkman, A.S. J. Gen. Physiol. (2001) [Pubmed]
 
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