SEARCH

SEARCH BY CITATION

Literature Cited

  • 1
    Riordan JR. CFTR function and prospects for therapy. Annu Rev Biochem 2008; 77: 701726.
  • 2
    Di A, Brown ME, Deriy LV, Li C, Szeto FL, Chen Y, Huang P, Tong J, Naren AP, Bindokas V, et al. CFTR regulates phagosome acidification in macrophages and alters bactericidal activity. Nat Cell Biol 2006; 8: 933944.
  • 3
    Mueller C, Braag SA, Keeler A, Hodges C, Drumm M, Flotte TR. Lack of cystic fibrosis transmembrane conductance regulator in CD3+ lymphocytes leads to aberrant cytokine secretion and hyperinflammatory adaptive immune responses. Am J Respir Cell Mol Biol 2011; 44: 922929.
  • 4
    Zielenski J. Genotype and phenotype in cystic fibrosis. Respiration 2000; 67: 117133.
  • 5
    Van Goor F, Hadida S, Grootenhuis PD, Burton B, Stack JH, Straley KS, Decker CJ, Miller M, McCartney J, Olson ER, et al. Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809. Proc Natl Acad Sci U S A 2011; 108: 1884318848.
  • 6
    Van Goor F, Hadida S, Grootenhuis PD, Burton B, Cao D, Neuberger T, Turnbull A, Singh A, Joubran J, Hazlewood A, et al. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc Natl Acad Sci U S A 2009; 106: 1882518830.
  • 7
    Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Drevinek P, Griese M, McKone EF, Wainwright CE, Konstan MW, et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 2011; 365: 16631672.
  • 8
    Moran O, Zegarra-Moran, O. On the measurement of the functional properties of the CFTR. J Cyst Fibros 2008; 7: 483494.
  • 9
    Galietta LJ, Haggie PM, Verkman AS. Green fluorescent protein-based halide indicators with improved chloride and iodide affinities. FEBS Lett 2001; 499: 220224.
  • 10
    Jayaraman S, Haggie P, Wachter RM, Remington SJ, Verkman AS. Mechanism and cellular applications of a green fluorescent protein-based halide sensor. J Biol Chem 2000; 275: 60476050.
  • 11
    Verkman AS, Galietta LJ. Chloride channels as drug targets. Nat Rev Drug Discov 2009; 8: 153171.
  • 12
    O'Ryan L, Rimington T, Cant N, Ford RC. Expression and purification of the cystic fibrosis transmembrane conductance regulator protein in Saccharomyces cerevisiae. J Vis Exp 2012; e3860.
  • 13
    Lukacs GL, Mohamed A, Kartner N, Chang XB, Riordan JR, Grinstein S. Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP. EMBO J 1994; 13: 60766086.
  • 14
    Beekman JM, Bakema JE, van der LJ, Tops B, Hinten M, vanVugt M, van de Winkel JG, Leusen JH. Modulation of FcgammaRI (CD64) ligand binding by blocking peptides of periplakin. J Biol Chem 2004; 279: 3387533881.
  • 15
    Beekman JM, Verhagen LP, Geijsen N, Coffer PJ. Regulation of myelopoiesis through syntenin-mediated modulation of IL-5 receptor output. Blood 2009; 114: 39173927.
  • 16
    Szymczak AL, Workman CJ, Wang Y, Vignali KM, Dilioglou S, Vanin EF, Vignali DA. Correction of multi-gene deficiency in vivo using a single ‘self-cleaving’ 2A peptide-based retroviral vector. Nat Biotechnol 2004; 22: 589594.
  • 17
    Shcherbo D, Murphy CS, Ermakova GV, Solovieva EA, Chepurnykh TV, Shcheglov AS, Verkhusha VV, Pletnev VZ, Hazelwood KL, Roche PM, et al. Far-red fluorescent tags for protein imaging in living tissues. Biochem J 2009; 418: 567574.
  • 18
    Nagai T, Ibata K, Park ES, Kubota M, Mikoshiba K, Miyawaki A. A variant of yellow fluorescent protein with fast and efficient maturation for cell-biological applications. Nat Biotechnol 2002; 20: 8790.
  • 19
    Beekman JM, Vervoort SJ, Dekkers F, van Vessem ME, Vendelbosch S, Brugulat-Panes A, vanLoosdregt J, Braat AK, Coffer PJ. Syntenin-mediated regulation of Sox4 proteasomal degradation modulates transcriptional output. Oncogene 2012; 31: 26882679.
  • 20
    van Meegen MA, Terheggen-Lagro SW, van der Ent CK, Beekman JM. CFTR expression analysis in human nasal epithelial cells by flow cytometry. PLoS One 2011; 6: e27658.
  • 21
    Gregory RJ, Cheng SH, Rich DP, Marshall J, Paul S, Hehir K, Ostedgaard L, Klinger KW, Welsh MJ, Smith AE. Expression and characterization of the cystic fibrosis transmembrane conductance regulator. Nature 1990; 347: 382386.
  • 22
    de Nooijer RA, Nobel JM, Arets HG, Bot AG, van Berkhout FT, de Rijke YB, de Jonge HR, Bronsveld I. Assessment of CFTR function in homozygous R117H-7T subjects. J Cyst Fibros 2011; 10: 326332.
  • 23
    Lee RJ, Foskett JK. cAMP-activated Ca2+ signaling is required for CFTR-mediated serous cell fluid secretion in porcine and human airways. J Clin Invest 2010; 120: 31373148.
  • 24
    Rich DP, Anderson MP, Gregory RJ, Cheng SH, Paul S, Jefferson DM, McCann JD, Klinger KW, Smith AE, Welsh MJ. Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells. Nature 1990; 347: 358363.
  • 25
    Jayaraman S, Teitler L, Skalski B, Verkman AS. Long-wavelength iodide-sensitive fluorescent indicators for measurement of functional CFTR expression in cells. Am J Physiol 1999; 277: C1008C1018.
  • 26
    Van Goor F, Straley KS, Cao D, Gonzalez J, Hadida S, Hazlewood A, Joubran J, Knapp T, Makings LR, Miller M, et al. Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules. Am J Physiol Lung Cell Mol Physiol 2006; 290: L1117L1130.
  • 27
    Gonzalez JE, Oades K, Leychkis Y, Harootunian A, Negulescu PA. Cell-based assays and instrumentation for screening ion-channel targets. Drug Discov Today 1999; 4: 431439.
  • 28
    Kuner T, Augustine GJ. A genetically encoded ratiometric indicator for chloride: Capturing chloride transients in cultured hippocampal neurons. Neuron 2000; 27: 447459.
  • 29
    Markova O, Mukhtarov M, Real E, Jacob Y, Bregestovski P. Genetically encoded chloride indicator with improved sensitivity. J Neurosci Methods 2008; 170: 6776.
  • 30
    Huang P, Stroffekova K, Cuppoletti J, Mahanty SK, Scarborough GA. Functional expression of the cystic fibrosis transmembrane conductance regulator in yeast. Biochim Biophys Acta 1996; 1281: 8090.