Related Content
Search Google Scholar for:
|
|
Science 8 September 1989: Vol. 245. no. 4922, pp. 1066 - 1073 DOI: 10.1126/science.2475911
|
|
Articles
Science, Vol 245, Issue 4922, 1066-1073
Copyright © 1989 by American Association for the Advancement of Science
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.
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
- Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770.
- F. Van Goor, S. Hadida, P. D. J. Grootenhuis, B. Burton, D. Cao, T. Neuberger, A. Turnbull, A. Singh, J. Joubran, A. Hazlewood, et al. (2009)
PNAS
106, 18825-18830
| Abstract »
| Full Text »
| PDF »
- Loss of TMEM16A Causes a Defect in Epithelial Ca2+-dependent Chloride Transport.
- J. Ousingsawat, J. R. Martins, R. Schreiber, J. R. Rock, B. D. Harfe, and K. Kunzelmann (2009)
J. Biol. Chem.
284, 28698-28703
| Abstract »
| Full Text »
| PDF »
- Mechanisms for Rescue of Correctable Folding Defects in CFTR{Delta}F508.
- D. E. Grove, M. F.N. Rosser, H. Y. Ren, A. P. Naren, and D. M. Cyr (2009)
Mol. Biol. Cell
20, 4059-4069
| Abstract »
| Full Text »
| PDF »
- Association of cholesterol oxidation and abnormalities in fatty acid metabolism in cystic fibrosis.
- L. Iuliano, R. Monticolo, G. Straface, S. Zullo, F. Galli, M. Boaz, and S. Quattrucci (2009)
Am. J. Clinical Nutrition
90, 477-484
| Abstract »
| Full Text »
| PDF »
- Pivotal Advance: Expansion of small sputum macrophages in CF: failure to express MARCO and mannose receptors.
- A. K. A. Wright, S. Rao, S. Range, C. Eder, T. P. J. Hofer, M. Frankenberger, L. Kobzik, C. Brightling, J. Grigg, and L. Ziegler-Heitbrock (2009)
J. Leukoc. Biol.
86, 479-489
| Abstract »
| Full Text »
| PDF »
- Interactions of Burkholderia cenocepacia and other Burkholderia cepacia complex bacteria with epithelial and phagocytic cells.
- M. S. Saldias and M. A. Valvano (2009)
Microbiology
155, 2809-2817
| Abstract »
| Full Text »
| PDF »
- Defective Acid Sphingomyelinase Pathway with Pseudomonas aeruginosa Infection in Cystic Fibrosis.
- H. Yu, Y. H. Zeidan, B. X. Wu, R. W. Jenkins, T. R. Flotte, Y. A. Hannun, and I. Virella-Lowell (2009)
Am. J. Respir. Cell Mol. Biol.
41, 367-375
| Abstract »
| Full Text »
| PDF »
- Influence of the Duplication of CFTR Exon 9 and Its Flanking Sequences on Diagnosis of Cystic Fibrosis Mutations.
- A. El-Seedy, T. Dudognon, F. Bilan, M.-C. Pasquet, M.-P. Reboul, A. Iron, A. Kitzis, and V. Ladeveze (2009)
J. Mol. Diagn.
11, 488-493
| Abstract »
| Full Text »
| PDF »
- Application of rate-equilibrium free energy relationship analysis to nonequilibrium ion channel gating mechanisms.
- L. Csanady (2009)
J. Gen. Physiol.
134, 129-136
| Abstract »
| Full Text »
| PDF »
- AMP-activated protein kinase phosphorylation of the R domain inhibits PKA stimulation of CFTR.
- J D. King Jr., A. C. Fitch, J. K. Lee, J. E. McCane, D.-O. D. Mak, J. K. Foskett, and K. R. Hallows (2009)
Am J Physiol Cell Physiol
297, C94-C101
| Abstract »
| Full Text »
| PDF »
- Revisiting the Role of Cystic Fibrosis Transmembrane Conductance Regulator and Counterion Permeability in the pH Regulation of Endocytic Organelles.
- H. Barriere, M. Bagdany, F. Bossard, T. Okiyoneda, G. Wojewodka, D. Gruenert, D. Radzioch, and G. L. Lukacs (2009)
Mol. Biol. Cell
20, 3125-3141
| Abstract »
| Full Text »
| PDF »
- A Small-Molecule Modulator Interacts Directly with {Delta}Phe508-CFTR to Modify Its ATPase Activity and Conformational Stability.
- L. Wellhauser, P. K. Chiaw, S. Pasyk, C. Li, M. Ramjeesingh, and C. E. Bear (2009)
Mol. Pharmacol.
75, 1430-1438
| Abstract »
| Full Text »
| PDF »
- The cystic fibrosis transmembrane conductance regulator in reproductive health and disease.
- H. C. Chan, Y. C. Ruan, Q. He, M. H. Chen, H. Chen, W. M. Xu, W. Y. Chen, C. Xie, X. H. Zhang, and Z. Zhou (2009)
J. Physiol.
587, 2187-2195
| Abstract »
| Full Text »
| PDF »
- Gating of the CFTR Cl\#8722; channel by ATP-driven nucleotide-binding domain dimerisation.
- T.-C. Hwang and D. N. Sheppard (2009)
J. Physiol.
587, 2151-2161
| Abstract »
| Full Text »
| PDF »
- Eradication of early Pseudomonas infection in cystic fibrosis.
- T. Lee (2009)
Chronic Respiratory Disease
6, 99-107
| Abstract »
| PDF »
- State-dependent modulation of CFTR gating by pyrophosphate.
- M.-F. Tsai, H. Shimizu, Y. Sohma, M. Li, and T.-C. Hwang (2009)
J. Gen. Physiol.
133, 405-419
| Abstract »
| Full Text »
| PDF »
- Genetic Modifiers Play a Substantial Role in Diabetes Complicating Cystic Fibrosis.
- S. M. Blackman, S. Hsu, L. L. Vanscoy, J. M. Collaco, S. E. Ritter, K. Naughton, and G. R. Cutting (2009)
J. Clin. Endocrinol. Metab.
94, 1302-1309
| Abstract »
| Full Text »
| PDF »
- Salmeterol Restores Secretory Functions in Cystic Fibrosis Airway Submucosal Gland Serous Cells.
- F. Delavoie, M. Molinari, M. Milliot, J.-M. Zahm, C. Coraux, J. Michel, and G. Balossier (2009)
Am. J. Respir. Cell Mol. Biol.
40, 388-397
| Abstract »
| Full Text »
| PDF »
- Management of Gene Promoter Mutations in Molecular Diagnostics.
- K. M. K. de Vooght, R. van Wijk, and W. W. van Solinge (2009)
Clin. Chem.
55, 698-708
| Abstract »
| Full Text »
| PDF »
- Cooperative Assembly and Misfolding of CFTR Domains In Vivo.
- K. Du and G. L. Lukacs (2009)
Mol. Biol. Cell
20, 1903-1915
| Abstract »
| Full Text »
| PDF »
- Mechanistic Insight into Control of CFTR by AMPK.
- P. Kongsuphol, D. Cassidy, B. Hieke, K. J. Treharne, R. Schreiber, A. Mehta, and K. Kunzelmann (2009)
J. Biol. Chem.
284, 5645-5653
| Abstract »
| Full Text »
| PDF »
- Detergent binding explains anomalous SDS-PAGE migration of membrane proteins.
- A. Rath, M. Glibowicka, V. G. Nadeau, G. Chen, and C. M. Deber (2009)
PNAS
106, 1760-1765
| Abstract »
| Full Text »
| PDF »
- ATP hydrolysis-driven gating in cystic fibrosis transmembrane conductance regulator.
- D. Muallem and P. Vergani (2009)
Phil Trans R Soc B
364, 247-255
| Abstract »
| Full Text »
| PDF »
- Burkholderia cepacia complex: epithelial cell-pathogen confrontations and potential for therapeutic intervention.
- S. McClean and M. Callaghan (2009)
J. Med. Microbiol.
58, 1-12
| Abstract »
| Full Text »
| PDF »
- Genetic basis of HDL variation in 129/SvImJ and C57BL/6J mice: importance of testing candidate genes in targeted mutant mice.
- Z. Su, X. Wang, S.-W. Tsaih, A. Zhang, A. Cox, S. Sheehan, and B. Paigen (2009)
J. Lipid Res.
50, 116-125
| Abstract »
| Full Text »
| PDF »
- Spiperone, identified through compound screening, activates calcium-dependent chloride secretion in the airway.
- L. Liang, K. MacDonald, E. M. Schwiebert, P. L. Zeitlin, and W. B. Guggino (2009)
Am J Physiol Cell Physiol
296, C131-C141
| Abstract »
| Full Text »
| PDF »
- Sequence-specific Retention and Regulated Integration of a Nascent Membrane Protein by the Endoplasmic Reticulum Sec61 Translocon.
- D. Pitonzo, Z. Yang, Y. Matsumura, A. E. Johnson, and W. R. Skach (2009)
Mol. Biol. Cell
20, 685-698
| Abstract »
| Full Text »
| PDF »
- Mutations at the Signature Sequence of CFTR Create a Cd2+-gated Chloride Channel.
- X. Wang, S. G. Bompadre, M. Li, and T.-C. Hwang (2008)
J. Gen. Physiol.
133, 69-77
| Abstract »
| Full Text »
| PDF »
- Burkholderia cenocepacia-induced delay of acidification and phagolysosomal fusion in cystic fibrosis transmembrane conductance regulator (CFTR)-defective macrophages.
- J. Lamothe and M. A. Valvano (2008)
Microbiology
154, 3825-3834
| Abstract »
| Full Text »
| PDF »
- PKC phosphorylation modulates PKA-dependent binding of the R domain to other domains of CFTR.
- G. Seavilleklein, N. Amer, A. Evagelidis, F. Chappe, T. Irvine, J. W. Hanrahan, and V. Chappe (2008)
Am J Physiol Cell Physiol
295, C1366-C1375
| Abstract »
| Full Text »
| PDF »
- Assembly and Misassembly of Cystic Fibrosis Transmembrane Conductance Regulator: Folding Defects Caused by Deletion of F508 Occur Before and After the Calnexin-dependent Association of Membrane Spanning Domain (MSD) 1 and MSD2.
- M. F. N. Rosser, D. E. Grove, L. Chen, and D. M. Cyr (2008)
Mol. Biol. Cell
19, 4570-4579
| Abstract »
| Full Text »
| PDF »
- Three-dimensional Reconstruction of Human Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel Revealed an Ellipsoidal Structure with Orifices beneath the Putative Transmembrane Domain.
- K. Mio, T. Ogura, M. Mio, H. Shimizu, T.-C. Hwang, C. Sato, and Y. Sohma (2008)
J. Biol. Chem.
283, 30300-30310
| Abstract »
| Full Text »
| PDF »
- Regulation of CFTR Trafficking by Its R Domain.
- C. M. Lewarchik, K. W. Peters, J. Qi, and R. A. Frizzell (2008)
J. Biol. Chem.
283, 28401-28412
| Abstract »
| Full Text »
| PDF »
- Processing Mutations Disrupt Interactions between the Nucleotide Binding and Transmembrane Domains of P-glycoprotein and the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
- T. W. Loo, M. C. Bartlett, and D. M. Clarke (2008)
J. Biol. Chem.
283, 28190-28197
| Abstract »
| Full Text »
| PDF »
- Anemia in Cystic Fibrosis: Incidence, Mechanisms, and Association With Pulmonary Function and Vitamin Deficiency.
- A. von Drygalski and J. Biller (2008)
Nutr Clin Pract
23, 557-563
| Abstract »
| Full Text »
| PDF »
- Vasoactive Intestinal Peptide Increases Cystic Fibrosis Transmembrane Conductance Regulator Levels in the Apical Membrane of Calu-3 Cells through a Protein Kinase C-Dependent Mechanism.
- F. Chappe, M. E. Loewen, J. W. Hanrahan, and V. Chappe (2008)
J. Pharmacol. Exp. Ther.
327, 226-238
| Abstract »
| Full Text »
| PDF »
- Animal models of chronic lung infection with Pseudomonas aeruginosa: useful tools for cystic fibrosis studies.
- I Kukavica-Ibrulj and R C Levesque (2008)
Lab Anim
42, 389-412
| Abstract »
| Full Text »
| PDF »
- Disruption of the CFTR Gene Produces a Model of Cystic Fibrosis in Newborn Pigs.
- C. S. Rogers, D. A. Stoltz, D. K. Meyerholz, L. S. Ostedgaard, T. Rokhlina, P. J. Taft, M. P. Rogan, A. A. Pezzulo, P. H. Karp, O. A. Itani, et al. (2008)
Science
321, 1837-1841
| Abstract »
| Full Text »
| PDF »
- Mapping of Interdomain Interfaces Required for the Functional Architecture of Yor1p, a Eukaryotic ATP-binding Cassette (ABC) Transporter.
- S. Pagant, E. Y. Brovman, J. J. Halliday, and E. A. Miller (2008)
J. Biol. Chem.
283, 26444-26451
| Abstract »
| Full Text »
| PDF »
- A mechanism accounting for the low cellular level of linoleic acid in cystic fibrosis and its reversal by DHA.
- M. R. Al-Turkmani, C. Andersson, R. Alturkmani, W. Katrangi, J. E. Cluette-Brown, S. D. Freedman, and M. Laposata (2008)
J. Lipid Res.
49, 1946-1954
| Abstract »
| Full Text »
| PDF »
- Validation of High-Resolution DNA Melting Analysis for Mutation Scanning of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gene.
- M.-P. Audrezet, A. Dabricot, C. Le Marechal, and C. Ferec (2008)
J. Mol. Diagn.
10, 424-434
| Abstract »
| Full Text »
| PDF »
- Chemotoxicity of doxorubicin and surface expression of P-glycoprotein (MDR1) is regulated by the Pseudomonas aeruginosa toxin Cif.
- S. Ye, D. P. MacEachran, J. W. Hamilton, G. A. O'Toole, and B. A. Stanton (2008)
Am J Physiol Cell Physiol
295, C807-C818
| Abstract »
| Full Text »
| PDF »
- Cell culture models demonstrate that CFTR dysfunction leads to defective fatty acid composition and metabolism.
- C. Andersson, M. R. Al-Turkmani, J. E. Savaille, R. Alturkmani, W. Katrangi, J. E. Cluette-Brown, M. M. Zaman, M. Laposata, and S. D. Freedman (2008)
J. Lipid Res.
49, 1692-1700
| Abstract »
| Full Text »
| PDF »
- A systematic analysis of intronic sequences downstream of 5' splice sites reveals a widespread role for U-rich motifs and TIA1/TIAL1 proteins in alternative splicing regulation.
- I. Aznarez, Y. Barash, O. Shai, D. He, J. Zielenski, L.-C. Tsui, J. Parkinson, B. J. Frey, J. M. Rommens, and B. J. Blencowe (2008)
Genome Res.
18, 1247-1258
| Abstract »
| Full Text »
| PDF »
- Cif Is Negatively Regulated by the TetR Family Repressor CifR.
- D. P. MacEachran, B. A. Stanton, and G. A. O'Toole (2008)
Infect. Immun.
76, 3197-3206
| Abstract »
| Full Text »
| PDF »
- The role of chloride anion and CFTR in killing of Pseudomonas aeruginosa by normal and CF neutrophils.
- R. G. Painter, R. W. Bonvillain, V. G. Valentine, G. A. Lombard, S. G. LaPlace, W. M. Nauseef, and G. Wang (2008)
J. Leukoc. Biol.
83, 1345-1353
| Abstract »
| Full Text »
| PDF »
- Pathophysiological basis of liver disease in cystic fibrosis employing a {Delta}F508 mouse model.
- F. Freudenberg, A. L. Broderick, B. B. Yu, M. R. Leonard, J. N. Glickman, and M. C. Carey (2008)
Am J Physiol Gastrointest Liver Physiol
294, G1411-G1420
| Abstract »
| Full Text »
| PDF »
- Cystic Fibrosis Transmembrane Conductance Regulator Controls Lung Proteasomal Degradation and Nuclear Factor-{kappa}B Activity in Conditions of Oxidative Stress.
- E. Boncoeur, T. Roque, E. Bonvin, V. Saint-Criq, M. Bonora, A. Clement, O. Tabary, A. Henrion-Caude, and J. Jacquot (2008)
Am. J. Pathol.
172, 1184-1194
| Abstract »
| Full Text »
| PDF »
- Genetic Analysis of Sporadic and Familial Interstitial Pneumonia.
- D. A. Schwartz (2008)
Proceedings of the ATS
5, 343-347
| Abstract »
| Full Text »
| PDF »
- CLC-0 and CFTR: Chloride Channels Evolved From Transporters.
- T.-Y. Chen and T.-C. Hwang (2008)
Physiol Rev
88, 351-387
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- Translation of Genetic Discoveries into Clinical Therapies.
- D. G. Hackam and A. S. Hackam (2008)
Ann Intern Med
148, 246-247
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- Cystic fibrosis.
- J. C Davies, E. W F W Alton, and A. Bush (2007)
BMJ
335, 1255-1259
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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)
Clin. Chem.
53, 1891-1898
| Abstract »
| Full Text »
| PDF »
- 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)
PNAS
104, 16365-16370
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- Protein interactions and disease: computational approaches to uncover the etiology of diseases.
- M. G. Kann (2007)
Brief Bioinform
8, 333-346
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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)
Mol. Biol. Cell
18, 3398-3413
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- Patterns of GI disease in adulthood associated with mutations in the CFTR gene.
- M. Wilschanski and P. R Durie (2007)
Gut
56, 1153-1163
| Full Text »
| PDF »
- Improved maturation of CFTR by an ER export signal.
- M. W. Wendeler, O. Nufer, and H.-P. Hauri (2007)
FASEB J
21, 2352-2358
| Abstract »
| Full Text »
| PDF »
- 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
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
- 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
| 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
| Abstract »
| Full Text »
| PDF »
- 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
| Full Text »
| PDF »
- Acute renal failure in people with cystic fibrosis.
- K. W Southern (2007)
Thorax
62, 472-473
| Full Text »
| PDF »
- 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
| Abstract »
| Full Text »
| PDF »
|
|