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Science 8 September 1989:
Vol. 245. no. 4922, pp. 1066 - 1073
DOI: 10.1126/science.2475911
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Articles

Science, Vol 245, Issue 4922, 1066-1073
Copyright © 1989 by American Association for the Advancement of Science

articles

Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA

Riordan JR, JM Rommens, B Kerem, N Alon, R Rozmahel, Z Grzelczak, J Zielenski, S Lok, N Plavsic, JL Chou, and al. et

Department of Biochemistry, Hospital for Sick Children, Toronto, Ontario, Canada.

Overlapping complementary DNA clones were isolated from epithelial cell libraries with a genomic DNA segment containing a portion of the putative cystic fibrosis (CF) locus, which is on chromosome 7. Transcripts, approximately 6500 nucleotides in size, were detectable in the tissues affected in patients with CF. The predicted protein consists of two similar motifs, each with (i) a domain having properties consistent with membrane association and (ii) a domain believed to be involved in ATP (adenosine triphosphate) binding. A deletion of three base pairs that results in the omission of a phenylalanine residue at the center of the first predicted nucleotide-binding domain was detected in CF patients.


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S. Ye, D. P. MacEachran, J. W. Hamilton, G. A. O'Toole, and B. A. Stanton (2008)
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Cell culture models demonstrate that CFTR dysfunction leads to defective fatty acid composition and metabolism.
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Pathophysiological basis of liver disease in cystic fibrosis employing a {Delta}F508 mouse model.
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Am J Physiol Gastrointest Liver Physiol 294, G1411-G1420
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Am. J. Pathol. 172, 1184-1194
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Genetic Analysis of Sporadic and Familial Interstitial Pneumonia.
D. A. Schwartz (2008)
Proceedings of the ATS 5, 343-347
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CLC-0 and CFTR: Chloride Channels Evolved From Transporters.
T.-Y. Chen and T.-C. Hwang (2008)
Physiol Rev 88, 351-387
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State-dependent Access of Anions to the Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel Pore.
M. Fatehi and P. Linsdell (2008)
J. Biol. Chem. 283, 6102-6109
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Preclinical Evidence that Sildenafil and Vardenafil Activate Chloride Transport in Cystic Fibrosis.
B. Lubamba, H. Lecourt, J. Lebacq, P. Lebecque, H. De Jonge, P. Wallemacq, and T. Leal (2008)
Am. J. Respir. Crit. Care Med. 177, 506-515
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Mechanism of G551D-CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) Potentiation by a High Affinity ATP Analog.
S. G. Bompadre, M. Li, and T.-C. Hwang (2008)
J. Biol. Chem. 283, 5364-5369
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PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X nonsense allele in a CF mouse model.
M. Du, X. Liu, E. M. Welch, S. Hirawat, S. W. Peltz, and D. M. Bedwell (2008)
PNAS 105, 2064-2069
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Translation of Genetic Discoveries into Clinical Therapies.
D. G. Hackam and A. S. Hackam (2008)
Ann Intern Med 148, 246-247
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8-iso-PGE2 Stimulates Anion Efflux from Airway Epithelial Cells via the EP4 Prostanoid Receptor.
A. P. Joy and E. A. Cowley (2008)
Am. J. Respir. Cell Mol. Biol. 38, 143-152
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Structural Analog of Sildenafil Identified as a Novel Corrector of the F508del-CFTR Trafficking Defect.
R. Robert, G. W. Carlile, C. Pavel, N. Liu, S. M. Anjos, J. Liao, Y. Luo, D. Zhang, D. Y. Thomas, and J. W. Hanrahan (2008)
Mol. Pharmacol. 73, 478-489
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State-dependent Inhibition of Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channels by a Novel Peptide Toxin.
M. D. Fuller, C. H. Thompson, Z.-R. Zhang, C. S. Freeman, E. Schay, G. Szakacs, E. Bakos, B. Sarkadi, D. McMaster, R. J. French, et al. (2007)
J. Biol. Chem. 282, 37545-37555
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Cystic fibrosis.
J. C Davies, E. W F W Alton, and A. Bush (2007)
BMJ 335, 1255-1259
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Myosin VI and its interacting protein LMTK2 regulate tubule formation and transport to the endocytic recycling compartment.
M. V. Chibalina, M. N. J. Seaman, C. C. Miller, J. Kendrick-Jones, and F. Buss (2007)
J. Cell Sci. 120, 4278-4288
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Molecular Proximity of Cystic Fibrosis Transmembrane Conductance Regulator and Epithelial Sodium Channel Assessed by Fluorescence Resonance Energy Transfer.
B. K. Berdiev, E. Cormet-Boyaka, A. Tousson, Y. J. Qadri, H. M. J. Oosterveld-Hut, J. S. Hong, P. A. Gonzales, C. M. Fuller, E. J. Sorscher, G. L. Lukacs, et al. (2007)
J. Biol. Chem. 282, 36481-36488
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Biogenesis of {gamma}-secretase early in the secretory pathway.
J. Kim, B. Kleizen, R. Choy, G. Thinakaran, S. S. Sisodia, and R. W. Schekman (2007)
J. Cell Biol. 179, 951-963
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Cellular Localization and Activity of Ad-Delivered GFP-CFTR in Airway Epithelial and Tracheal Cells.
O. Granio, C. Norez, K. J. D. Ashbourne Excoffon, P. H. Karp, M. Lusky, F. Becq, P. Boulanger, J. Zabner, and S.-S. Hong (2007)
Am. J. Respir. Cell Mol. Biol. 37, 631-639
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A monoclonal antibody prevents aggregation of the NBD1 domain of the cystic fibrosis transmembrane conductance regulator.
V. Lovato, C. Roesli, J. Ahlskog, J. Scheuermann, and D. Neri (2007)
Protein Eng. Des. Sel. 20, 607-614
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Correctors Promote Maturation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-processing Mutants by Binding to the Protein.
Y. Wang, T. W. Loo, M. C. Bartlett, and D. M. Clarke (2007)
J. Biol. Chem. 282, 33247-33251
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Expression and function of cystic fibrosis transmembrane conductance regulator in rat intrapulmonary arteries.
R. Robert, J-P. Savineau, C. Norez, F. Becq, and C. Guibert (2007)
Eur. Respir. J. 30, 857-864
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Scanning the Cystic Fibrosis Transmembrane Conductance Regulator Gene Using High-Resolution DNA Melting Analysis.
J. Montgomery, C. T. Wittwer, J. O. Kent, and L. Zhou (2007)
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Chimeric constructs endow the human CFTR Cl channel with the gating behavior of murine CFTR.
T. S. Scott-Ward, Z. Cai, E. S. Dawson, A. Doherty, A. Carina Da Paula, H. Davidson, D. J. Porteous, B. J. Wainwright, M. D. Amaral, D. N. Sheppard, et al. (2007)
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Pancreatic phenotype in infants with cystic fibrosis identified by mutation screening.
M. Cipolli, C. Castellani, B. Wilcken, J. Massie, K. McKay, M. Gruca, A. Tamanini, M. B. Assael, and K. Gaskin (2007)
Arch. Dis. Child. 92, 842-846
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Processing and function of CFTR-{Delta}F508 are species-dependent.
L. S. Ostedgaard, C. S. Rogers, Q. Dong, C. O. Randak, D. W. Vermeer, T. Rokhlina, P. H. Karp, and M. J. Welsh (2007)
PNAS 104, 15370-15375
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Live Cell FRET Microscopy: HOMO- AND HETERODIMERIZATION OF TWO HUMAN PEROXISOMAL ABC TRANSPORTERS, THE ADRENOLEUKODYSTROPHY PROTEIN (ALDP, ABCD1) AND PMP70 (ABCD3).
M. Hillebrand, S. E. Verrier, A. Ohlenbusch, A. Schafer, H.-D. Soling, F. S. Wouters, and J. Gartner (2007)
J. Biol. Chem. 282, 26997-27005
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Protein Kinase C Phosphorylation Disrupts Na+/H+ Exchanger Regulatory Factor 1 Autoinhibition and Promotes Cystic Fibrosis Transmembrane Conductance Regulator Macromolecular Assembly.
J. Li, P. I. Poulikakos, Z. Dai, J. R. Testa, D. J. E. Callaway, and Z. Bu (2007)
J. Biol. Chem. 282, 27086-27099
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Protein interactions and disease: computational approaches to uncover the etiology of diseases.
M. G. Kann (2007)
Brief Bioinform 8, 333-346
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Discovery of {alpha}-Aminoazaheterocycle-Methylglyoxal Adducts as a New Class of High-Affinity Inhibitors of Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channels.
C. Routaboul, C. Norez, P. Melin, M.-C. Molina, B. Boucherle, F. Bossard, S. Noel, R. Robert, C. Gauthier, F. Becq, et al. (2007)
J. Pharmacol. Exp. Ther. 322, 1023-1035
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Inhibiting Endoplasmic Reticulum (ER)-associated Degradation of Misfolded Yor1p Does Not Permit ER Export Despite the Presence of a Diacidic Sorting Signal.
S. Pagant, L. Kung, M. Dorrington, M. C.S. Lee, and E. A. Miller (2007)
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Myosin Vb Is Required for Trafficking of the Cystic Fibrosis Transmembrane Conductance Regulator in Rab11a-specific Apical Recycling Endosomes in Polarized Human Airway Epithelial Cells.
A. Swiatecka-Urban, L. Talebian, E. Kanno, S. Moreau-Marquis, B. Coutermarsh, K. Hansen, K. H. Karlson, R. Barnaby, R. E. Cheney, G. M. Langford, et al. (2007)
J. Biol. Chem. 282, 23725-23736
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Advancing Outcome Measures for the New Era of Drug Development in Cystic Fibrosis.
N. Mayer-Hamblett, B. W. Ramsey, and R. A. Kronmal (2007)
Proceedings of the ATS 4, 370-377
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The Pseudomonas aeruginosa Secreted Protein PA2934 Decreases Apical Membrane Expression of the Cystic Fibrosis Transmembrane Conductance Regulator.
D. P. MacEachran, S. Ye, J. M. Bomberger, D. A. Hogan, A. Swiatecka-Urban, B. A. Stanton, and G. A. O'Toole (2007)
Infect. Immun. 75, 3902-3912
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Correlation of Chest Radiograph Pattern With Genotype, Age, and Gender in Adult Cystic Fibrosis: A Single-Center Study.
V. Kaza, M. F. Katz, S. Cumming, A. E. Frost, and Z. Safdar (2007)
Chest 132, 569-574
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Patterns of GI disease in adulthood associated with mutations in the CFTR gene.
M. Wilschanski and P. R Durie (2007)
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The inexhaustible mouse nose. Focus on "Olfactory epithelia exhibit progressive functional and morphological defects in CF mice".
J. J. Wine (2007)
Am J Physiol Cell Physiol 293, C537-C539
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Quorum-sensing blockade as a strategy for enhancing host defences against bacterial pathogens.
T. Bjarnsholt and M. Givskov (2007)
Phil Trans R Soc B 362, 1213-1222
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A Comparative Study of Five Technologically Diverse CFTR Testing Platforms.
M. A. Johnson, M. J. Yoshitomi, and C. S. Richards (2007)
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   Abstract »    Full Text »    PDF »
Design, Development, Validation, and Use of Synthetic Nucleic Acid Controls for Diagnostic Purposes and Application to Cystic Fibrosis Testing.
T. M. Christensen, M. Jama, V. Ponek, E. Lyon, J. A. Wilson, M. L. Hoffmann, and B. A. Bejjani (2007)
J. Mol. Diagn. 9, 315-319
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Genetics of Gestational Diabetes Mellitus and Type 2 Diabetes.
R. M. Watanabe, M. H. Black, A. H. Xiang, H. Allayee, J. M. Lawrence, and T. A. Buchanan (2007)
Diabetes Care 30, S134-S140
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Acute renal failure in people with cystic fibrosis.
K. W Southern (2007)
Thorax 62, 472-473
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VCP/p97 AAA-ATPase Does Not Interact with the Endogenous Wild-Type Cystic Fibrosis Transmembrane Conductance Regulator.
R. F. Goldstein, A. Niraj, T. P. Sanderson, L. S. Wilson, A. Rab, H. Kim, Z. Bebok, and J. F. Collawn (2007)
Am. J. Respir. Cell Mol. Biol. 36, 706-714
   Abstract »    Full Text »    PDF »
Airway Nitric Oxide in Patients With Cystic Fibrosis Is Associated With Pancreatic Function, Pseudomonas Infection, and Polyunsaturated Fatty Acids.
C. Keen, A.-C. Olin, A. Edentoft, E. Gronowitz, and B. Strandvik (2007)
Chest 131, 1857-1864
   Abstract »    Full Text »    PDF »
Inhibition of CFTR Cl- channel function caused by enzymatic hydrolysis of sphingomyelin.
Y. Ramu, Y. Xu, and Z. Lu (2007)
PNAS 104, 6448-6453
   Abstract »    Full Text »    PDF »
Regulatory interactions of N1303K-CFTR and ENaC in Xenopus oocytes: evidence that chloride transport is not necessary for inhibition of ENaC.
L. Suaud, W. Yan, M. D. Carattino, A. Robay, T. R. Kleyman, and R. C. Rubenstein (2007)
Am J Physiol Cell Physiol 292, C1553-C1561
   Abstract »    Full Text »    PDF »
G551D and G1349D, Two CF-associated Mutations in the Signature Sequences of CFTR, Exhibit Distinct Gating Defects.
S. G. Bompadre, Y. Sohma, M. Li, and T.-C. Hwang (2007)
J. Gen. Physiol. 129, 285-298
   Abstract »    Full Text »    PDF »
Targeting CAL as a Negative Regulator of {Delta}F508-CFTR Cell-Surface Expression: AN RNA INTERFERENCE AND STRUCTURE-BASED MUTAGENETIC APPROACH.
M. Wolde, A. Fellows, J. Cheng, A. Kivenson, B. Coutermarsh, L. Talebian, K. Karlson, A. Piserchio, D. F. Mierke, B. A. Stanton, et al. (2007)
J. Biol. Chem. 282, 8099-8109
   Abstract »    Full Text »    PDF »
{Delta}F508 Mutation Results in Impaired Gastric Acid Secretion.
S. M. Sidani, P. Kirchhoff, T. Socrates, L. Stelter, E. Ferreira, C. Caputo, K. E. Roberts, R. L. Bell, M. E. Egan, and J. P. Geibel (2007)
J. Biol. Chem. 282, 6068-6074
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