Removal of Multiple Arginine-Framed Trafficking Signals Overcomes Misprocessing of ΔF508 CFTR Present in Most Patients with Cystic Fibrosis

Aleksandrov, 1998, Regulation of CFTR ion channel gating by MgATP, FEBS Lett., 431, 97, 10.1016/S0014-5793(98)00713-3 Brown, 1996, Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein, Cell Stress Chaperones, 1, 117, 10.1379/1466-1268(1996)001<0117:CCCTMP>2.3.CO;2 Chang, 1993, Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all ten PKA consensus phosphorylation sites, J. Biol. Chem., 268, 11304, 10.1016/S0021-9258(18)82125-1 Dalemans, 1991, Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation, Nature, 354, 526, 10.1038/354526a0 Denning, 1992, Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive, Nature, 358, 761, 10.1038/358761a0 Drumm, 1991, Chloride conductance expressed by ΔF508 and other mutant CFTRs in Xenopus oocytes, Science, 254, 1797, 10.1126/science.1722350 Gaynor, 1998, COPI in ER/Golgi and intra-Golgi transport, Biochim. Biophys. Acta, 1404, 33, 10.1016/S0167-4889(98)00045-7 Gilbert, 1998, Delta F508 CFTR localizes in the endoplasmic reticulum-Golgi intermediate compartment in cystic fibrosis cells, Exp. Cell Res., 242, 144, 10.1006/excr.1998.4101 Jensen, 1995, Multiple proteolytic systems, including the proteasome, contribute to CFTR processing, Cell, 83, 129, 10.1016/0092-8674(95)90241-4 Jiang, 1998, Partial restoration of cAMP-stimulated CFTR chloride channel activity in ΔF508 cells by deoxyspergualin, Am. J. Physiol., 275, C171, 10.1152/ajpcell.1998.275.1.C171 Kartner, 1992, Mislocalization of delta F508 CFTR in cystic fibrosis sweat gland, Nat. Genet., 1, 321, 10.1038/ng0892-321 Loo, 1998, Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome, EMBO J., 17, 6879, 10.1093/emboj/17.23.6879 Marshall, 1994, Stoichiometry of recombinant cystic fibrosis transmembrane conductance regulator in epithelial cells and its functional reconstitution into cells in vitro, J. Biol. Chem., 269, 2987, 10.1016/S0021-9258(17)42037-0 Meacham, 1999, The Hdj-2/Hsc 70 chaperone pair facilitates early steps in CFTR biogenesis, EMBO J., 18, 1492, 10.1093/emboj/18.6.1492 Nishimura, 1997, A di-acidic signal required for selective export from the endoplasmic reticulum, Science, 277, 556, 10.1126/science.277.5325.556 Pind, 1994, Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator, J. Biol. Chem., 269, 12784, 10.1016/S0021-9258(18)99944-8 Prochiantz, 1998, Peptide nucleic acid smugglers, Nat. Biotechnol., 16, 819, 10.1038/nbt0998-819 Qu, 1996, Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway, J. Biol. Chem., 271, 7261, 10.1074/jbc.271.13.7261 Rothman, 1996, Protein sorting by transport vesicles, Science, 272, 227, 10.1126/science.272.5259.227 Sato, 1996, Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation, J. Biol. Chem., 271, 635, 10.1074/jbc.271.2.635 Seibert, 1997, Cystic fibrosis, J. Bioenerg. Biomembr., 29, 429, 10.1023/A:1022478822214 Teasdale, 1996, Signal-mediated sorting of membrane proteins between the endoplasmic reticulum and the golgi apparatus, Annu. Rev. Cell Dev. Biol., 12, 27, 10.1146/annurev.cellbio.12.1.27 Teem, 1993, Identification of revertants for the cystic fibrosis ΔF508 mutation using STE6-CFTR chimeras in yeast, Cell, 73, 335, 10.1016/0092-8674(93)90233-G Ward, 1995, Degradation of CFTR by the ubiquitin-proteasome pathway, Cell, 83, 121, 10.1016/0092-8674(95)90240-6 Yang, 1993, The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment, Proc. Natl. Acad. Sci. USA, 90, 9480, 10.1073/pnas.90.20.9480 Zerangue, 1999, A new ER trafficking signal regulates the subunit stoichiometry of plasma membrane KATP channels, Neuron, 22, 537, 10.1016/S0896-6273(00)80708-4 Zerhusen, 1999, A single conductance pore for chloride ions formed by two cystic fibrosis transmembrane conductance regulator molecules, J. Biol. Chem., 274, 7627, 10.1074/jbc.274.12.7627 Zhang, 1998, Limited proteolysis as a probe for arrested conformational maturation of delta F508 CFTR, Nat. Struct. Biol., 5, 180, 10.1038/nsb0398-180