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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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Brief Bioinform 8, 333-346
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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)
Gut 56, 1153-1163
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Improved maturation of CFTR by an ER export signal.
M. W. Wendeler, O. Nufer, and H.-P. Hauri (2007)
FASEB J 21, 2352-2358
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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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A Comparative Study of Five Technologically Diverse CFTR Testing Platforms.
M. A. Johnson, M. J. Yoshitomi, and C. S. Richards (2007)
J. Mol. Diagn. 9, 401-407
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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
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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
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Simulation of the Coupling between Nucleotide Binding and Transmembrane Domains in the ATP Binding Cassette Transporter BtuCD.
J. Sonne, C. Kandt, G. H. Peters, F. Y. Hansen, M. O. Jensen, and D. P. Tieleman (2007)
Biophys. J. 92, 2727-2734
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Inhibition of CFTR Cl- channel function caused by enzymatic hydrolysis of sphingomyelin.
Y. Ramu, Y. Xu, and Z. Lu (2007)
PNAS 104, 6448-6453
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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
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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
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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
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{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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Y. Wang, T. W. Loo, M. C. Bartlett, and D. M. Clarke (2007)
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F. Jouret, A. Bernard, C. Hermans, G. Dom, S. Terryn, T. Leal, P. Lebecque, J.-J. Cassiman, B. J. Scholte, H. R. de Jonge, et al. (2007)
J. Am. Soc. Nephrol. 18, 707-718
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Freedom of expression. Focus on "Endoplasmic reticulum stress and the unfolded protein response regulate cystic fibrosis transmembrane conductance regulator expression".
N. A. Bradbury (2007)
Am J Physiol Cell Physiol 292, C687-C688
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Strategies for Identifying Modifier Genes in Cystic Fibrosis.
M. P. Boyle (2007)
Proceedings of the ATS 4, 52-57
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Expression of ABCA3 in Developing Lung and Other Tissues.
M. T. Stahlman, V. Besnard, S. E. Wert, T. E. Weaver, S. Dingle, Y. Xu, K. von Zychlin, S. J. Olson, and J. A. Whitsett (2007)
J. Histochem. Cytochem. 55, 71-83
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Abnormal regulatory interactions of I148T-CFTR and the epithelial Na+ channel in Xenopus oocytes.
L. Suaud, W. Yan, and R. C. Rubenstein (2007)
Am J Physiol Cell Physiol 292, C603-C611
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Derlin-1 Promotes the Efficient Degradation of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) and CFTR Folding Mutants.
F. Sun, R. Zhang, X. Gong, X. Geng, P. F. Drain, and R. A. Frizzell (2006)
J. Biol. Chem. 281, 36856-36863
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Fucosylation in prokaryotes and eukaryotes.
B. Ma, J. L. Simala-Grant, and D. E. Taylor (2006)
Glycobiology 16, 158R-184R
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Positive Charges at the Intracellular Mouth of the Pore Regulate Anion Conduction in the CFTR Chloride Channel.
C. N. St. Aubin and P. Linsdell (2006)
J. Gen. Physiol. 128, 535-545
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Thermodynamics of CFTR Channel Gating: A Spreading Conformational Change Initiates an Irreversible Gating Cycle.
L. Csanady, A. C. Nairn, and D. C. Gadsby (2006)
J. Gen. Physiol. 128, 523-533
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Two novel missense and one novel nonsense CFTR mutations in Iranian males with congenital bilateral absence of the vas deferens.
R. Radpour, H. Gourabi, M. A.S. Gilani, A. V. Dizaj, M. Rezaee, and S. Mollamohamadi (2006)
Mol. Hum. Reprod. 12, 717-721
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Insertion of an Arginine Residue into the Transmembrane Segments Corrects Protein Misfolding.
T. W. Loo, M. C. Bartlett, and D. M. Clarke (2006)
J. Biol. Chem. 281, 29436-29440
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The Two ATP Binding Sites of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Play Distinct Roles in Gating Kinetics and Energetics.
Z. Zhou, X. Wang, H.-Y. Liu, X. Zou, M. Li, and T.-C. Hwang (2006)
J. Gen. Physiol. 128, 413-422
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Discovery of Pyrrolo[2,3-b]pyrazines Derivatives as Submicromolar Affinity Activators of Wild Type, G551D, and F508del Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channels.
S. Noel, C. Faveau, C. Norez, C. Rogier, Y. Mettey, and F. Becq (2006)
J. Pharmacol. Exp. Ther. 319, 349-359
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A New Insertion/Deletion of the Cystic Fibrosis Transmembrane Conductance Regulator Gene Accounts for 3.4% of Cystic Fibrosis Mutations in Sardinia: Implications for Population Screening.
V. Faa, P. P. Bettoli, M. Demurtas, M. Zanda, V. Ferri, A. Cao, and M. C. Rosatelli (2006)
J. Mol. Diagn. 8, 499-503
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De Novo Biosynthetic Profiling of High Abundance Proteins in Cystic Fibrosis Lung Epithelial Cells.
H. B. Pollard, O. Eidelman, C. Jozwik, W. Huang, M. Srivastava, X. D. Ji, B. McGowan, C. F. Norris, T. Todo, T. Darling, et al. (2006)
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I. R. Bates, B. Hebert, Y. Luo, J. Liao, A. I. Bachir, D. L. Kolin, P. W. Wiseman, and J. W. Hanrahan (2006)
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K. F.K. Ejendal, N. K. Diop, L. C. Schweiger, and C. A. Hrycyna (2006)
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   Abstract »    Full Text »    PDF »
The cystic fibrosis transmembrane conductance regulator (Cftr) modulates the timing of puberty in mice..
R Jin, C A Hodges, M L Drumm, and M R Palmert (2006)
J. Med. Genet. 43, e29
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Rescue of {Delta}F508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules.
F. Van Goor, K. S. Straley, D. Cao, J. Gonzalez, S. Hadida, A. Hazlewood, J. Joubran, T. Knapp, L. R. Makings, M. Miller, et al. (2006)
Am J Physiol Lung Cell Mol Physiol 290, L1117-L1130
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Calreticulin Negatively Regulates the Cell Surface Expression of Cystic Fibrosis Transmembrane Conductance Regulator.
K. Harada, T. Okiyoneda, Y. Hashimoto, K. Ueno, K. Nakamura, K. Yamahira, T. Sugahara, T. Shuto, I. Wada, M. A. Suico, et al. (2006)
J. Biol. Chem. 281, 12841-12848