Pharmacologic restoration of αδF508 CFTR-mediated chloride current

This publication has 46 references indexed in Scilit:

• Defective aquaporin-2 trafficking in nephrogenic diabetes insipidus and correction by chemical chaperones.
  Journal of Clinical Investigation, 1998
• Direct Activation of Cystic Fibrosis Transmembrane Conductance Regulator Channels by 8-Cyclopentyl-1,3-dipropylxanthine (CPX) and 1,3-Diallyl-8-cyclohexylxanthine (DAX)
  Published by Elsevier ,1998
• The Molecular Chaperone Hsc70 Assists the in VitroFolding of the N-terminal Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator
  Published by Elsevier ,1997
• 8-Cyclopentyl-1,3-dipropylxanthine and Other Xanthines Differentially Bind to the Wild-Type and ΔF508 Mutant First Nucleotide Binding Fold (NBF-1) Domains of the Cystic Fibrosis Transmembrane Conductance Regulator
  Biochemistry, 1997
• Correcting temperature-sensitive protein folding defects.
  Journal of Clinical Investigation, 1997
• A1 Receptor Antagonist 8-Cyclopentyl-1,3-dipropylxanthine Selectively Activates Chloride Efflux from Human Epithelial and Mouse Fibroblast Cell Lines Expressing the Cystic Fibrosis Transmembrane Regulator .DELTA.F508 Mutation
  Biochemistry, 1995
• Similar levels of mRNA from the W1282X and the delta F508 cystic fibrosis alleles, in nasal epithelial cells.
  Journal of Clinical Investigation, 1994
• The cystic fibrosis mutation (ΔF508) does not influence the chloride channel activity of CFTR
  Nature Genetics, 1993
• Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation
  Nature, 1991
• Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA
  Science, 1989