Regulatory insertion removal restores maturation, stability and function of DeltaF508 CFTR.

[1]  John F Hunt,et al.  Structures of a minimal human CFTR first nucleotide-binding domain as a monomer, head-to-tail homodimer, and pathogenic mutant. , 2010, Protein engineering, design & selection : PEDS.

[2]  S. Matalon,et al.  Functional stability of rescued delta F508 cystic fibrosis transmembrane conductance regulator in airway epithelial cells. , 2010, American journal of respiratory cell and molecular biology.

[3]  Jianping Wu,et al.  ATP-independent CFTR channel gating and allosteric modulation by phosphorylation , 2010, Proceedings of the National Academy of Sciences.

[4]  J. M. Sauder,et al.  Structure and dynamics of NBD1 from CFTR characterized using crystallography and hydrogen/deuterium exchange mass spectrometry. , 2010, Journal of molecular biology.

[5]  Julie D Forman-Kay,et al.  NMR evidence for differential phosphorylation‐dependent interactions in WT and ΔF508 CFTR , 2010, The EMBO journal.

[6]  D. Cyr,et al.  Mechanisms for rescue of correctable folding defects in CFTRDelta F508. , 2009, Molecular biology of the cell.

[7]  Isabelle Callebaut,et al.  Molecular models of the open and closed states of the whole human CFTR protein , 2009, Cellular and Molecular Life Sciences.

[8]  J. Riordan,et al.  Relationship between nucleotide binding and ion channel gating in cystic fibrosis transmembrane conductance regulator , 2009, The Journal of physiology.

[9]  G. Lukács,et al.  N-glycans are direct determinants of CFTR folding and stability in secretory and endocytic membrane traffic , 2009, The Journal of cell biology.

[10]  J. Riordan,et al.  Mg2+ -dependent ATP occlusion at the first nucleotide-binding domain (NBD1) of CFTR does not require the second (NBD2). , 2008, The Biochemical journal.

[11]  J. Riordan,et al.  Chemical and Biological Folding Contribute to Temperature‐Sensitive ΔF508 CFTR Trafficking , 2008, Traffic.

[12]  Nikolay V. Dokholyan,et al.  Multiple Membrane-Cytoplasmic Domain Contacts in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mediate Regulation of Channel Gating* , 2008, Journal of Biological Chemistry.

[13]  Feng Ding,et al.  Active Nuclear Receptors Exhibit Highly Correlated AF-2 Domain Motions , 2008, PLoS Comput. Biol..

[14]  J. Riordan,et al.  CFTR function and prospects for therapy. , 2008, Annual review of biochemistry.

[15]  Adrian W. R. Serohijos,et al.  Phenylalanine-508 mediates a cytoplasmic–membrane domain contact in the CFTR 3D structure crucial to assembly and channel function , 2008, Proceedings of the National Academy of Sciences.

[16]  Nikolay V. Dokholyan,et al.  Diminished Self-Chaperoning Activity of the ΔF508 Mutant of CFTR Results in Protein Misfolding , 2008, PLoS Comput. Biol..

[17]  M. Amaral,et al.  Solubilizing mutations used to crystallize one CFTR domain attenuate the trafficking and channel defects caused by the major cystic fibrosis mutation. , 2008, Chemistry & biology.

[18]  Ying Wang,et al.  Correctors Promote Maturation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-processing Mutants by Binding to the Protein* , 2007, Journal of Biological Chemistry.

[19]  D. Clarke,et al.  Additive effect of multiple pharmacological chaperones on maturation of CFTR processing mutants. , 2007, The Biochemical journal.

[20]  Feng Ding,et al.  Multiscale modeling of nucleosome dynamics. , 2007, Biophysical journal.

[21]  Andrei Aleksandrov,et al.  Domain interdependence in the biosynthetic assembly of CFTR. , 2007, Journal of molecular biology.

[22]  Andrei A. Aleksandrov,et al.  CFTR (ABCC7) is a hydrolyzable-ligand-gated channel , 2007, Pflügers Archiv - European Journal of Physiology.

[23]  John D. Venable,et al.  Hsp90 Cochaperone Aha1 Downregulation Rescues Misfolding of CFTR in Cystic Fibrosis , 2006, Cell.

[24]  James Rader,et al.  Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules. , 2006, American journal of physiology. Lung cellular and molecular physiology.

[25]  Feng Ding,et al.  Emergence of Protein Fold Families through Rational Design , 2006, PLoS Comput. Biol..

[26]  J. Riordan,et al.  F508del CFTR with two altered RXR motifs escapes from ER quality control but its channel activity is thermally sensitive. , 2006, Biochimica et biophysica acta.

[27]  J. Riordan,et al.  The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gating , 2006, The Journal of physiology.

[28]  Kai Du,et al.  Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening. , 2005, The Journal of clinical investigation.

[29]  J. M. Sauder,et al.  Impact of the ΔF508 Mutation in First Nucleotide-binding Domain of Human Cystic Fibrosis Transmembrane Conductance Regulator on Domain Folding and Structure* , 2005, Journal of Biological Chemistry.

[30]  A. Nairn,et al.  Functional Roles of Nonconserved Structural Segments in CFTR's NH2-terminal Nucleotide Binding Domain , 2005, The Journal of general physiology.

[31]  Kai Du,et al.  The ΔF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR , 2005, Nature Structural &Molecular Biology.

[32]  J. Ruysschaert,et al.  Mistargeted MRPΔF728 mutant is rescued by intracellular GSH , 2004 .

[33]  J. M. Sauder,et al.  Structure of nucleotide‐binding domain 1 of the cystic fibrosis transmembrane conductance regulator , 2004, The EMBO journal.

[34]  B. Papsin,et al.  Misfolding diverts CFTR from recycling to degradation: quality control at early endosomes , 2004 .

[35]  J. Ruysschaert,et al.  Mistargeted MRPdeltaF728 mutant is rescued by intracellular GSH. , 2004, FEBS letters.

[36]  T. Ma,et al.  Nanomolar Affinity Small Molecule Correctors of Defective ΔF508-CFTR Chloride Channel Gating* , 2003, Journal of Biological Chemistry.

[37]  S. Gullans,et al.  Mammalian Osmolytes and S-Nitrosoglutathione Promote ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Protein Maturation and Function* , 2003, Journal of Biological Chemistry.

[38]  A. Nairn,et al.  Prolonged Nonhydrolytic Interaction of Nucleotide with CFTR's NH2-terminal Nucleotide Binding Domain and its Role in Channel Gating , 2003, The Journal of general physiology.

[39]  L. Schmitt,et al.  Crystal structure of the nucleotide-binding domain of the ABC-transporter haemolysin B: identification of a variable region within ABC helical domains. , 2003, Journal of molecular biology.

[40]  T. Ma,et al.  Nanomolar affinity small molecule correctors of defective Delta F508-CFTR chloride channel gating. , 2003, The Journal of biological chemistry.

[41]  L. Gansheroff,et al.  Mutations in the Nucleotide Binding Domain 1 Signature Motif Region Rescue Processing and Functional Defects of Cystic Fibrosis Transmembrane Conductance Regulator ΔF508* , 2002, The Journal of Biological Chemistry.

[42]  D. Clarke,et al.  Introduction of the Most Common Cystic Fibrosis Mutation (ΔF508) into Human P-glycoprotein Disrupts Packing of the Transmembrane Segments* , 2002, The Journal of Biological Chemistry.

[43]  J. Riordan,et al.  The First Nucleotide Binding Domain of Cystic Fibrosis Transmembrane Conductance Regulator Is a Site of Stable Nucleotide Interaction, whereas the Second Is a Site of Rapid Turnover* , 2002, The Journal of Biological Chemistry.

[44]  J. Riordan,et al.  Differential Interactions of Nucleotides at the Two Nucleotide Binding Domains of the Cystic Fibrosis Transmembrane Conductance Regulator* , 2001, The Journal of Biological Chemistry.

[45]  Fei Wang,et al.  Deletion of phenylalanine 508 causes attenuated phosphorylation‐dependent activation of CFTR chloride channels , 2000, The Journal of physiology.

[46]  R. Frizzell,et al.  Rescue of Dysfunctional ΔF508-CFTR Chloride Channel Activity by IBMX , 1999, The Journal of Membrane Biology.

[47]  R. Frizzell,et al.  Rescue of dysfunctional deltaF508-CFTR chloride channel activity by IBMX. , 1999, The Journal of membrane biology.

[48]  J. Riordan,et al.  Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome , 1998, The EMBO journal.

[49]  H. Stanley,et al.  Discrete molecular dynamics studies of the folding of a protein-like model. , 1998, Folding & design.

[50]  E. Strickland,et al.  Localization and Suppression of a Kinetic Defect in Cystic Fibrosis Transmembrane Conductance Regulator Folding* , 1997, The Journal of Biological Chemistry.

[51]  H. Wakelee,et al.  Delta F508-CFTR channels: kinetics, activation by forskolin, and potentiation by xanthines. , 1996, The American journal of physiology.

[52]  Satoshi Omura,et al.  Degradation of CFTR by the ubiquitin-proteasome pathway , 1995, Cell.

[53]  J. Riordan,et al.  Multiple proteolytic systems, including the proteasome, contribute to CFTR processing , 1995, Cell.

[54]  J. Riordan,et al.  Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites. , 1993, The Journal of biological chemistry.

[55]  H. A. Berger,et al.  Identification of revertants for the cystic fibrosis ΔF508 mutation using STE6-CFTR chimeras in yeast , 1993, Cell.

[56]  Matthew P. Anderson,et al.  Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive , 1992, Nature.

[57]  Pascal Barbry,et al.  Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation , 1991, Nature.

[58]  R. Crystal,et al.  Altered chloride ion channel kinetics associated with the delta F508 cystic fibrosis mutation. , 1991, Nature.