John R RiordanSummaryAffiliation: Mayo Clinic Country: USA Publications
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Assembly of functional CFTR chloride channelsJohn R Riordan
Mayo Clinic College of Medicine, Scottsdale, Arizona, 85259, USA
Annu Rev Physiol 67:701-18. 2005..The biosynthetic processing of the nascent polypeptide leading to channel assembly involves transient interactions with numerous chaperones and enzymes on both sides of the endoplasmic reticulum membrane...- Nucleoside triphosphate pentose ring impact on CFTR gating and hydrolysisAndrei A Aleksandrov
Mayo Foundation and Mayo Clinic Scottsdale, S C Johnson Medical Research Center, 13400 E Shea Blvd, Scottsdale, AZ 85259, USA
FEBS Lett 518:183-8. 2002.... - 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 TurnoverLuba Aleksandrov
Mayo Foundation and Mayo Clinic Scottsdale, S. C. Johnson Medical Research Center, Scottsdale, Arizona 85259, USA
J Biol Chem 277:15419-25. 2002..This demonstration of NBD2 as the rapid nucleotide turnover site is consistent with the strong effect on channel gating kinetics of inactivation of this domain by mutagenesis... - ATP binding to the first nucleotide-binding domain of multidrug resistance protein MRP1 increases binding and hydrolysis of ATP and trapping of ADP at the second domainYue-Xian Hou
Mayo Foundation, S. C. Johnson Medical Research Center, Mayo Clinic Scottsdale, Scottsdale, Arizona 85259, USA
J Biol Chem 277:5110-9. 2002..The interactions between the two asymmetric NBDs of MRP1 protein may enhance the catalytic efficiency of the MRP1 protein and hence of its ATP-dependent transport of conjugated anions out of cells... - ATP binding to the first nucleotide binding domain of multidrug resistance-associated protein plays a regulatory role at low nucleotide concentration, whereas ATP hydrolysis at the second plays a dominant role in ATP-dependent leukotriene C4 transportRunying Yang
Mayo Foundation, S C Johnson Medical Research Center, Mayo Clinic Scottsdale, Scottsdale, Arizona 85259, USA
J Biol Chem 278:30764-71. 2003.... 
Functional analysis of the C-terminal boundary of the second nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator and structural implicationsMartina Gentzsch
Mayo Foundation and Mayo Clinic Scottsdale, S. C. Johnson Medical Research Center, 13400 E. Shea Blvd, Scottsdale, AZ 85259, USA
Biochem J 366:541-8. 2002....- ATP binding, not hydrolysis, at the first nucleotide-binding domain of multidrug resistance-associated protein MRP1 enhances ADP.Vi trapping at the second domainYue Xian Hou
S C Johnson Medical Research Center, Mayo Foundation, Mayo Clinic Scottsdale, Scottsdale, Arizona 85259, USA
J Biol Chem 278:3599-605. 2003.... - Role of N-linked oligosaccharides in the biosynthetic processing of the cystic fibrosis membrane conductance regulatorXiu Bao Chang
Mayo Clinic College of Medicine, Mayo Clinic Arizona, Scottsdale, AZ 85259, USA
J Cell Sci 121:2814-23. 2008..Surprisingly, the individual N-linked carbohydrates do not play equivalent roles and modulate the fate of the wild-type protein in different ways in its early biosynthetic pathway... - Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulatorMartina Gentzsch
Mayo Clinic College of Medicine, S C Johnson Medical Research Center, Department of Biochemistry and Molecular Biology, Mayo Clinic, Scottsdale, Arizona 85259, USA
Mol Biol Cell 15:2684-96. 2004..Importantly, however, the mutant protein can be rescued at the plasma membrane by Rab11 overexpression, proteasome inhibitors, or inhibition of Rab5-dependent endocytosis... - The PDZ-binding chloride channel ClC-3B localizes to the Golgi and associates with cystic fibrosis transmembrane conductance regulator-interacting PDZ proteinsMartina Gentzsch
Mayo Clinic Scottsdale, S C Johnson Medical Research Center, Scottsdale, Arizona 85259, USA
J Biol Chem 278:6440-9. 2003.... - The role of cystic fibrosis transmembrane conductance regulator phenylalanine 508 side chain in ion channel gatingLiying Cui
Department of Biochemistry and Biophysics, University of North Carolina, Chapel Hill, 27599, USA
J Physiol 572:347-58. 2006..Thus, despite the fact that this aromatic side chain is not essential for CFTR folding, it is important in the ion channel function... - CFTR (ABCC7) is a hydrolyzable-ligand-gated channelAndrei A Aleksandrov
Department of Biochemistry and Biophysics and Cystic Fibrosis Center, University of North Carolina, Chapel Hill, NC 27599, USA
Pflugers Arch 453:693-702. 2007..ATP hydrolysis is not required for either the opening or closing gating transitions but efficiently clears the ligand-binding site enabling a new gating cycle to be initiated... - Domain interdependence in the biosynthetic assembly of CFTRLiying Cui
Dept of Biochemistry and Biophysics and Cystic Fibrosis Center, University of North Carolina at Chapel Hill, NC 27599, USA
J Mol Biol 365:981-94. 2007..The dispensability of NBD2 in the folding of more N-terminal domains stands in contrast to the known hypersensitivity to proteolysis of NBD2 in the DeltaF508 protein... - Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel functionAdrian W R Serohijos
Department of Biochemistry and Biophysics, University of North Carolina, Chapel Hill, NC 27599, USA
Proc Natl Acad Sci U S A 105:3256-61. 2008.... - Computational studies reveal phosphorylation-dependent changes in the unstructured R domain of CFTRTamas Hegedus
Department of Biochemistry and Biophysics, University of North Carolina Chapel Hill, Chapel Hill, NC 27599, USA
J Mol Biol 378:1052-63. 2008.... - Purification and crystallization of the cystic fibrosis transmembrane conductance regulator (CFTR)Mark F Rosenberg
Department of Biomolecular Sciences, University of Manchester Institute of Science and Technology, Manchester M60 1QD, United Kingdom
J Biol Chem 279:39051-7. 2004..The hypothesis that the two conformations could represent the "open" and "closed" states of the channel is considered... 
Mg2+ -dependent ATP occlusion at the first nucleotide-binding domain (NBD1) of CFTR does not require the second (NBD2)Luba Aleksandrov
Department of Biochemistry Biophysics, University of North Carolina, Chapel Hill, NC 27599, USA
Biochem J 416:129-36. 2008..Occlusion occurred identically in a DeltaNBD2 construct, but not in purified recombinant NBD1, indicating that the process is dependent on the influence of regions of CFTR in addition to NBD1, but not NBD2...- Multiple membrane-cytoplasmic domain contacts in the cystic fibrosis transmembrane conductance regulator (CFTR) mediate regulation of channel gatingLihua He
Department of Biochemistry and Biophysics, University of North Carolina, Chapel Hill, North Carolina 27599, USA
J Biol Chem 283:26383-90. 2008..These results reinforce the idea that mediation of regulatory signals between cytoplasmic- and membrane-integrated domains of the CFTR channel apparently relies on an array of precise but highly dynamic interdomain structural joints... - Diminished self-chaperoning activity of the DeltaF508 mutant of CFTR results in protein misfoldingAdrian W R Serohijos
Department of Physics and Astronomy, University of North Carolina Chapel Hill, Chapel Hill, North Carolina, USA
PLoS Comput Biol 4:e1000008. 2008..The identified structural determinants of increased misfolding propensity of NBD1-DeltaF508 are essential information in correcting this pathogenic mutant... - CFTR function and prospects for therapyJohn R Riordan
Department of Biochemistry and Biophysics, Cystic Fibrosis Treatment and Research Center, School of Medicine, University of North Carolina at Chapel Hill, NC 27599, USA
Annu Rev Biochem 77:701-26. 2008..This review considers different therapeutic strategies that have arisen from knowledge of CFTR structure and function as well as its biosynthetic processing, intracellular trafficking, and turnover... - Misassembled mutant DeltaF508 CFTR in the distal secretory pathway alters cellular lipid traffickingMartina Gentzsch
Department of Cell and Developmental Biology and Cystic Fibrosis Research Center, University of North Carolina, Chapel Hill, NC 27599, USA
J Cell Sci 120:447-55. 2007..Thus, on escape from ER quality control, misassembled mutants of CFTR and MRP1 impair lipid homeostasis in endocytic compartments... - Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosisXiaodong Wang
Department of Cell Biology, The Scripps Research Institute, La Jolla, CA 92037, USA
Cell 127:803-15. 2006..The activity of cargo-associated chaperome components may be a common mechanism regulating folding for ER exit, providing a general framework for correction of misfolding disease... - F508del CFTR with two altered RXR motifs escapes from ER quality control but its channel activity is thermally sensitiveTamas Hegedus
Department of Biochemistry and Biophysics and Cystic Fibrosis T and R Center, UNC, 5011 Thurston Bowles Bldg, Chapel Hill, NC 27510 7248, USA
Biochim Biophys Acta 1758:565-72. 2006..Therefore effective molecular therapies developed to alleviate disease caused by F508del and probably other misprocessing mutants will require overcoming both their kinetic and steady-state impacts... - Stimulatory and inhibitory protein kinase C consensus sequences regulate the cystic fibrosis transmembrane conductance regulatorValerie Chappe
Department of Physiology, McGill University, Montreal, QC, Canada H3G 1Y6
Proc Natl Acad Sci U S A 101:390-5. 2004..These results identify functionally important PKC consensus sequences on CFTR and will facilitate studies of its convergent regulation by PKC and PKA... - The DeltaF508 mutation results in loss of CFTR function and mature protein in native human colonMarcus Mall
School of Medicine, University of North Carolina at Chapel Hill, NC 27599, USA
Gastroenterology 126:32-41. 2004..However, data with respect to the processing block of DeltaF508 protein in native epithelia are limited and conflicting... - Fluorescent modified phosphatidylcholine floppase activity of reconstituted multidrug resistance-associated protein MRP1Zhenhua Huang
National Laboratory of Biomacromolecules, Center for Molecular Biology, Institute of Biophysics, Chinese Academy of Sciences, Beijing 100101, China
Biochim Biophys Acta 1660:155-63. 2004..Taken together, the purified and reconstituted MRP1 protein possesses the ability to flop NBD-PC from outer to inner leaflet of the proteoliposomes... - Cystic fibrosis transmembrane conductance regulator degradation depends on the lectins Htm1p/EDEM and the Cdc48 protein complex in yeastAndreas Gnann
Institut fur Biochemie, Universitat Stuttgart, 70569 Stuttgart, Germany
Mol Biol Cell 15:4125-35. 2004..These proteins also were found to be necessary for ERAD of a mutated yeast "relative" of CFTR, Pdr5(*)p... - Novel regulation of cystic fibrosis transmembrane conductance regulator (CFTR) channel gating by external chlorideAngela M Wright
Institute of Cell and Molecular Biosciences, University of Newcastle upon Tyne, Framlington Place, Newcastle upon Tyne, NE2 4HH, United Kingdom
J Biol Chem 279:41658-63. 2004..Physiologically, our data have implications for current models of epithelial HCO(3)(-) secretion and for the control of pH at epithelial cell surfaces... - Determination of CFTR chloride channel activity and pharmacology using radiotracer flux methodsCaroline Norez
Institut de Physiologie et Biologie Cellulaires CNRS UMR 6187, , 40 Avenue du Recteur Pineau, Poitiers, 86022 France
J Cyst Fibros 3:119-21. 2004..Moreover, they have proved especially valuable in the search for new drugs to treat cystic fibrosis... - Intermediate structural states involved in MRP1-mediated drug transport. Role of glutathioneLiliana Manciu
Structure and Function of Biological Membranes Center of Structural Biology and Bioinformatics, Free University of Brussels, B 1050 Brussels, Belgium
J Biol Chem 278:3347-56. 2003..Binding of a non-transported drug affects the dynamic changes occurring during ATP binding and hydrolysis and restricts the movement of the drug and its release... - Bcr (breakpoint cluster region) protein binds to PDZ-domains of scaffold protein PDZK1 and vesicle coat protein Mint3Emily K Malmberg
Department of Medical Biochemistry, Goteborg University, Medicinaregatan 9A, 413 90 Gothenburg, Sweden
J Cell Sci 117:5535-41. 2004..Hence these findings extend the interactions and likely signaling impact of Bcr in epithelia from the cytosol to at least these two membrane compartments... - Non-conventional trafficking of the cystic fibrosis transmembrane conductance regulator through the early secretory pathwayJin San Yoo
Department of Cell and Molecular Biology, The Scripps Research Institute, La Jolla, California 92037, USA
J Biol Chem 277:11401-9. 2002.... - Characterization of wild-type and deltaF508 cystic fibrosis transmembrane regulator in human respiratory epitheliaSilvia M Kreda
Cystic Fibrosis Pulmonary Research and Treatment Center, School of Medicine, The University of North Carolina at Chapel Hill, Chapel Hill, NC 27599 7248, USA
Mol Biol Cell 16:2154-67. 2005..In sum, our data demonstrate that WT CFTR is predominantly expressed in ciliated cells, and deltaF508 CFTR pathogenesis in native tissues, like heterologous cells, reflects loss of normal protein processing... - SERCA pump inhibitors do not correct biosynthetic arrest of deltaF508 CFTR in cystic fibrosisBarbara R Grubb
Cystic Fibrosis Pulmonary Research and Treatment Center, 7011 Thurston Bowles Bldg, CB 7248, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599 7248, USA
Am J Respir Cell Mol Biol 34:355-63. 2006.... 
COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit codeXiaodong Wang
Department of Cell Biology, The Scripps Research Institute, La Jolla, CA 92037, USA
J Cell Biol 167:65-74. 2004..We propose that the di-acidic exit code plays a key role in linking CFTR to the COPII coat machinery and is the primary defect responsible for CF in DeltaF508-expressing patients...