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Gene Review

CFTR  -  cystic fibrosis transmembrane conductance...

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Disease relevance of CFTR


High impact information on CFTR


Chemical compound and disease context of CFTR

  • A growing body of evidence suggests that there are marked similarities in the properties of the cAMP-dependent chloride channels in heart and cystic fibrosis transmembrane regulator (CFTR) chloride channels found in airway epithelia or in cells expressing the CFTR gene product [10].

Biological context of CFTR

  • Comparison of the amino acid sequence of NBD1 of human CFTR with the deduced sequence of the rabbit heart PCR product indicated 98% identity [10].
  • Glibenclamide markedly inhibited CFTR Cl- currents in a voltage-independent manner at 22 degrees C, with estimated IC50 values of 12.5 and 11.0 mumol/L at +50 and -100 mV, respectively [1].
  • Endocytosis of wt-CFTR and CFTR-DeltaTRL did not differ [11].
  • Pulse-chase studies in combination with domain-selective cell surface biotinylation revealed that newly synthesized wt-CFTR and CFTR-DeltaTRL were targeted equally to the apical and basolateral membranes in a nonpolarized fashion [11].
  • The relationship between cell proliferation, Cl- secretion, and renal cyst growth: a study using CFTR inhibitors [4].

Anatomical context of CFTR

  • Whole-cell patch-clamp techniques were used to compare the effects of glibenclamide on CFTR Cl- currents in guinea pig ventricular myocytes, swelling-activated Cl- currents in guinea pig atrial myocytes, and Ca(2+)-activated Cl- currents in canine ventricular myocytes [1].
  • We tested the hypothesis that the PDZ interacting domain regulates sorting of CFTR from the Golgi to the apical plasma membrane [11].
  • In contrast, when 3T3 fibroblasts stably expressing CFTR were stimulated with cAMP agonists, cell ATP levels decreased [12].
  • Using a series of defined chimeric and truncated proteins expressed in a reticulocyte lysate system, we have identified two topogenic determinants encoded within the first (TM1) and second (TM2) membrane-spanning segments of CFTR [13].
  • Disruption of the microtubular cytoskeleton with colchicine did not affect cAMP-stimulated Cl- secretion or GFP-CFTR expression in the apical membrane [14].

Associations of CFTR with chemical compounds

  • These results suggest that glibenclamide, an inhibitor of cardiac CFTR Cl- channels, also inhibits swelling-activated and Ca(2+)-activated Cl- channels at higher concentrations [1].
  • We conclude that CFTR chloride channels are expressed in heart and are responsible for the observed cAMP-dependent chloride conductance [10].
  • We conclude that cAMP stimulates CFTR-mediated Cl- secretion in MDCK type I cells by activating channels resident in the apical plasma membrane [14].
  • Mutating charged residues Glu92 and Lys95 to alanine improved TM1 signal sequence activity as well as the ability of TM1 to independently direct CFTR N terminus topology [13].
  • Iodide efflux measurements indicate that CFTR expression confers a plasma membrane anion conductance that is responsive to stimulation by cAMP [3].

Other interactions of CFTR


Analytical, diagnostic and therapeutic context of CFTR

  • Assaying for possible changes in CFTR by immunoprecipitation and immunocytochemical localization revealed that CFTR appeared as an immature 140-kDa form intracellularly in conventional cultures [16].
  • Immunofluorescence microscopic studies revealed intracellular colocalization of mucins and the cystic fibrosis transmembrane conductance regulator (CFTR) [17].
  • Interestingly, at low multiplicity of infection, we observed FSK-stimulated insertion of M2901/CFTR into the apical PM, whereas at higher M2-901/CFTR expression levels, no increase in surface expression was detected using indirect immunofluorescence [5].
  • Immunoelectron microscopy of unstimulated and FSK-stimulated cells confirmed the M2-901/CFTR redistribution to the PM upon FSK stimulation and demonstrates that the apically inserted M2-901/CFTR originates from a population of subapical vesicles [5].
  • The presence of CFTR was confirmed by immunoprecipitation followed by Western blotting [18].


  1. Inhibitory effects of glibenclamide on cystic fibrosis transmembrane regulator, swelling-activated, and Ca(2+)-activated Cl- channels in mammalian cardiac myocytes. Yamazaki, J., Hume, J.R. Circ. Res. (1997) [Pubmed]
  2. Commensal bacteria increase invasion of intestinal epithelium by Salmonella enterica serovar Typhi. Lyczak, J.B. Infect. Immun. (2003) [Pubmed]
  3. Functional expression and apical localization of the cystic fibrosis transmembrane conductance regulator in MDCK I cells. Mohamed, A., Ferguson, D., Seibert, F.S., Cai, H.M., Kartner, N., Grinstein, S., Riordan, J.R., Lukacs, G.L. Biochem. J. (1997) [Pubmed]
  4. The relationship between cell proliferation, Cl- secretion, and renal cyst growth: a study using CFTR inhibitors. Li, H., Findlay, I.A., Sheppard, D.N. Kidney Int. (2004) [Pubmed]
  5. Forskolin-induced apical membrane insertion of virally expressed, epitope-tagged CFTR in polarized MDCK cells. Howard, M., Jiang, X., Stolz, D.B., Hill, W.G., Johnson, J.A., Watkins, S.C., Frizzell, R.A., Bruton, C.M., Robbins, P.D., Weisz, O.A. Am. J. Physiol., Cell Physiol. (2000) [Pubmed]
  6. Epithelial transport in polycystic kidney disease. Sullivan, L.P., Wallace, D.P., Grantham, J.J. Physiol. Rev. (1998) [Pubmed]
  7. CFTR as a cAMP-dependent regulator of sodium channels. Stutts, M.J., Canessa, C.M., Olsen, J.C., Hamrick, M., Cohn, J.A., Rossier, B.C., Boucher, R.C. Science (1995) [Pubmed]
  8. A PDZ-interacting domain in CFTR is an apical membrane polarization signal. Moyer, B.D., Denton, J., Karlson, K.H., Reynolds, D., Wang, S., Mickle, J.E., Milewski, M., Cutting, G.R., Guggino, W.B., Li, M., Stanton, B.A. J. Clin. Invest. (1999) [Pubmed]
  9. CFTR Cl- channel and CFTR-associated ATP channel: distinct pores regulated by common gates. Sugita, M., Yue, Y., Foskett, J.K. EMBO J. (1998) [Pubmed]
  10. Expression of cystic fibrosis transmembrane regulator Cl- channels in heart. Levesque, P.C., Hart, P.J., Hume, J.R., Kenyon, J.L., Horowitz, B. Circ. Res. (1992) [Pubmed]
  11. PDZ domain interaction controls the endocytic recycling of the cystic fibrosis transmembrane conductance regulator. Swiatecka-Urban, A., Duhaime, M., Coutermarsh, B., Karlson, K.H., Collawn, J., Milewski, M., Cutting, G.R., Guggino, W.B., Langford, G., Stanton, B.A. J. Biol. Chem. (2002) [Pubmed]
  12. Effect of cAMP on intracellular and extracellular ATP content of Cl- -secreting epithelia and 3T3 fibroblasts. Takahashi, T., Matsushita, K., Welsh, M.J., Stokes, J.B. J. Biol. Chem. (1994) [Pubmed]
  13. Co- and posttranslational translocation mechanisms direct cystic fibrosis transmembrane conductance regulator N terminus transmembrane assembly. Lu, Y., Xiong, X., Helm, A., Kimani, K., Bragin, A., Skach, W.R. J. Biol. Chem. (1998) [Pubmed]
  14. Membrane trafficking of the cystic fibrosis gene product, cystic fibrosis transmembrane conductance regulator, tagged with green fluorescent protein in madin-darby canine kidney cells. Moyer, B.D., Loffing, J., Schwiebert, E.M., Loffing-Cueni, D., Halpin, P.A., Karlson, K.H., Ismailov, I.I., Guggino, W.B., Langford, G.M., Stanton, B.A. J. Biol. Chem. (1998) [Pubmed]
  15. Evidence against the acidification hypothesis in cystic fibrosis. Gibson, G.A., Hill, W.G., Weisz, O.A. Am. J. Physiol., Cell Physiol. (2000) [Pubmed]
  16. Improved oxygenation promotes CFTR maturation and trafficking in MDCK monolayers. Bebök, Z., Tousson, A., Schwiebert, L.M., Venglarik, C.J. Am. J. Physiol., Cell Physiol. (2001) [Pubmed]
  17. Mucous granule exocytosis and CFTR expression in gallbladder epithelium. Kuver, R., Klinkspoor, J.H., Osborne, W.R., Lee, S.P. Glycobiology (2000) [Pubmed]
  18. Characterization of the ion transport responses to ADH in the MDCK-C7 cell line. Lahr, T.F., Record, R.D., Hoover, D.K., Hughes, C.L., Blazer-Yost, B.L. Pflugers Arch. (2000) [Pubmed]
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