Science 8 February 1991: Vol. 251. no. 4994, pp. 679 - 682 DOI: 10.1126/science.1704151

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Articles

The Cystic Fibrosis Transmembrane Conductance Regulator and Chloride-Dependent Ion Fluxes of Ovine Vocal Fold Epithelium.

C. Leydon, K. V. Fisher, and D. Lodewyck-Falciglia (2009)
J Speech Lang Hear Res 52, 745-754
 |  Abstract »  |  Full Text »  |  PDF »

Amiloride: Still a Viable Treatment Option in Cystic Fibrosis?.

F. Ratjen and A. Bush (2008)
Am. J. Respir. Crit. Care Med. 178, 1191-1192
 |  Full Text »  |  PDF »

Multiplex Ligation-Dependent Probe Amplification Identification of Whole Exon and Single Nucleotide Deletions in the CFTR Gene of Hispanic Individuals with Cystic Fibrosis.

I. Schrijver, K. Rappahahn, L. Pique, M. Kharrazi, and L.-J. Wong (2008)
J. Mol. Diagn. 10, 368-375
 |  Abstract »  |  Full Text »  |  PDF »

Dynamic Regulation of Cystic Fibrosis Transmembrane Conductance Regulator by Competitive Interactions of Molecular Adaptors.

J. H. Lee, W. Richter, W. Namkung, K. H. Kim, E. Kim, M. Conti, and M. G. Lee (2007)
J. Biol. Chem. 282, 10414-10422
 |  Abstract »  |  Full Text »  |  PDF »

Profile and factors determining outcome in a cohort of cystic fibrosis patients seen at the aga khan university hospital, karachi, pakistan..

U. Shah, T. Moatter, and Z. A. Bhutta (2006)
J Trop Pediatr 52, 132-135
 |  Abstract »  |  Full Text »  |  PDF »

Influence of Cystic Fibrosis Transmembrane Conductance Regulator on Gene Expression in Response to Pseudomonas aeruginosa Infection of Human Bronchial Epithelial Cells.

N. Reiniger, J. K. Ichikawa, and G. B. Pier (2005)
Infect. Immun. 73, 6822-6830
 |  Abstract »  |  Full Text »  |  PDF »

Diagnostic Testing by CFTR Gene Mutation Analysis in a Large Group of Hispanics: Novel Mutations and Assessment of a Population-Specific Mutation Spectrum.

I. Schrijver, S. Ramalingam, R. Sankaran, S. Swanson, C. L.M. Dunlop, S. Keiles, R. B. Moss, J. Oehlert, P. Gardner, E. R. Wassman, et al. (2005)
J. Mol. Diagn. 7, 289-299
 |  Abstract »  |  Full Text »  |  PDF »

Release of ATP from retinal pigment epithelial cells involves both CFTR and vesicular transport.

D. Reigada and C. H. Mitchell (2005)
Am J Physiol Cell Physiol 288, C132-C140
 |  Abstract »  |  Full Text »  |  PDF »

Potentiation of effect of PKA stimulation of Xenopus CFTR by activation of PKC: role of NBD2.

Y. Chen, B. Button, G. A. Altenberg, and L. Reuss (2004)
Am J Physiol Cell Physiol 287, C1436-C1444
 |  Abstract »  |  Full Text »  |  PDF »

Reversal of cystic fibrosis phenotype in a cultured {Delta}508 cystic fibrosis transmembrane conductance regulator cell line by oligonucleotide insertion.

P. C. Zamecnik, M. K. Raychowdhury, D. R. Tabatadze, and H. F. Cantiello (2004)
PNAS 101, 8150-8155
 |  Abstract »  |  Full Text »  |  PDF »

Characteristic Multiorgan Pathology of Cystic Fibrosis in a Long-Living Cystic Fibrosis Transmembrane Regulator Knockout Murine Model.

P. R. Durie, G. Kent, M. J. Phillips, and C. A. Ackerley (2004)
Am. J. Pathol. 164, 1481-1493
 |  Abstract »  |  Full Text »  |  PDF »

Plasmodium falciparum-activated Chloride Channels Are Defective in Erythrocytes from Cystic Fibrosis Patients.

P. Verloo, C. H. M. Kocken, A. Van der Wel, B. C. Tilly, B. M. Hogema, M. Sinaasappel, A. W. Thomas, and H. R. De Jonge (2004)
J. Biol. Chem. 279, 10316-10322
 |  Abstract »  |  Full Text »  |  PDF »

Inhibitory Regulation of Cystic Fibrosis Transmembrane Conductance Regulator Anion-transporting Activities by Shank2.

J. Y. Kim, W. Han, W. Namkung, J. H. Lee, K. H. Kim, H. Shin, E. Kim, and M. G. Lee (2004)
J. Biol. Chem. 279, 10389-10396
 |  Abstract »  |  Full Text »  |  PDF »

Gene expression profile analysis of 4-phenylbutyrate treatment of IB3-1 bronchial epithelial cell line demonstrates a major influence on heat-shock proteins.

J. M. Wright, P. L. Zeitlin, L. Cebotaru, S. E. Guggino, and W. B. Guggino (2004)
Physiol Genomics 16, 204-211
 |  Abstract »  |  Full Text »  |  PDF »

Revisiting Cystic Fibrosis Transmembrane Conductance Regulator Structure and Function.

J. W. Hanrahan and M.-A. Wioland (2004)
Proceedings of the ATS 1, 17-21
 |  Abstract »  |  Full Text »  |  PDF »

Independence of apical Cl-/HCO3- exchange and anion conductance in duodenal HCO3- secretion.

S. Spiegel, M. Phillipper, H. Rossmann, B. Riederer, M. Gregor, and U. Seidler (2003)
Am J Physiol Gastrointest Liver Physiol 285, G887-G897
 |  Abstract »  |  Full Text »  |  PDF »

A haplotype-based molecular analysis of CFTR mutations associated with respiratory and pancreatic diseases.

J. H. Lee, J. H. Choi, W. Namkung, J. W. Hanrahan, J. Chang, S. Y. Song, S. W. Park, D. S. Kim, J.-H. Yoon, Y. Suh, et al. (2003)
Hum. Mol. Genet. 12, 2321-2332
 |  Abstract »  |  Full Text »  |  PDF »

A Multicenter Study of the Effect of Solution Temperature on Nasal Potential Difference Measurements.

M. P. Boyle, M. Diener-West, L. Milgram, M. Knowles, C. Foy, P. Zeitlin, and T. Standaert (2003)
Chest 124, 482-489
 |  Abstract »  |  Full Text »  |  PDF »

Disruptive effects of anion secretion inhibitors on airway mucus morphology in isolated perfused pig lung.

L. Trout, M. I Townsley, A. L Bowden, and S. T Ballard (2003)
J. Physiol. 549, 845-853
 |  Abstract »  |  Full Text »  |  PDF »

Measurement of Nasal Potential Difference in Mild Asthmatics.

N. C. Chung, B. Illek, J. H. Widdicombe, and H. Fischer (2003)
Chest 123, 1467-1471
 |  Abstract »  |  Full Text »  |  PDF »

Ion channels in secretory granules of the pancreas and their role in exocytosis and release of secretory proteins.

F. Thevenod (2002)
Am J Physiol Cell Physiol 283, C651-C672
 |  Abstract »  |  Full Text »  |  PDF »

Evolutionary Analyses of ABC Transporters of Dictyostelium discoideum.

C. Anjard, the Dictyostelium Sequencing Consortium, and W. F. Loomis (2002)
Eukaryot. Cell 1, 643-652
 |  Abstract »  |  Full Text »  |  PDF »

Lung Infections Associated with Cystic Fibrosis.

J. B. Lyczak, C. L. Cannon, and G. B. Pier (2002)
Clin. Microbiol. Rev. 15, 194-222
 |  Abstract »  |  Full Text »  |  PDF »

{micro}2 Binding Directs the Cystic Fibrosis Transmembrane Conductance Regulator to the Clathrin-mediated Endocytic Pathway.

K. M. Weixel and N. A. Bradbury (2001)
J. Biol. Chem. 276, 46251-46259
 |  Abstract »  |  Full Text »  |  PDF »

Examining basal chloride transport using the nasal potential difference response in a murine model.

K. G. Brady, T. J. Kelley, and M. L. Drumm (2001)
Am J Physiol Lung Cell Mol Physiol 281, L1173-L1179
 |  Abstract »  |  Full Text »  |  PDF »

Voltage-dependent flickery block of an open cystic fibrosis transmembrane conductance regulator (CFTR) channel pore.

Z. Zhou, S. Hu, and T.-C. Hwang (2001)
J. Physiol. 532, 435-448
 |  Abstract »  |  Full Text »  |  PDF »

Mechanisms of chloride transport in thymic lymphocytes.

D. Stakisaitis, M. S. Lapointe, and D. Batlle (2001)
Am J Physiol Renal Physiol 280, F314-F324
 |  Abstract »  |  Full Text »  |  PDF »

Generation and phenotype of cell lines derived from CF and non-CF mice that carry the H-2Kb-tsA58 transgene.

M. Takacs-Jarrett, W. E. Sweeney, E. D. Avner, and C. U. Cotton (2001)
Am J Physiol Cell Physiol 280, C228-C236
 |  Abstract »  |  Full Text »  |  PDF »

Structural determinants for activation and block of CFTR-mediated chloride currents by apigenin.

B. Illek, M. E. Lizarzaburu, V. Lee, M. H. Nantz, M. J. Kurth, and H. Fischer (2000)
Am J Physiol Cell Physiol 279, C1838-C1846
 |  Abstract »  |  Full Text »  |  PDF »

The {{Delta}}F508 Mutation in the Cystic Fibrosis Transmembrane Conductance Regulator Alters Control of Essential Fatty Acid Utilization in Epithelial Cells.

F. N. Bhura-Bandali, M. Suh, S. F. P. Man, and M. T. Clandinin (2000)
J. Nutr. 130, 2870-2875
 |  Abstract »  |  Full Text »  |  PDF »

Expression of nucleotide-regulated Cl- currents in CF and normal mouse tracheal epithelial cell lines.

E. J. Thomas, S. E. Gabriel, M. Makhlina, S. P. Hardy, and M. I. Lethem (2000)
Am J Physiol Cell Physiol 279, C1578-C1586
 |  Abstract »  |  Full Text »  |  PDF »

Evidence for cystic fibrosis transmembrane conductance regulator-dependent sodium reabsorption in kidney, using Cftrtm2cammice.

J D Kibble, A M Neal, W H Colledge, R Green, and C J Taylor (2000)
J. Physiol. 526, 27-34
 |  Abstract »  |  Full Text »  |  PDF »

cAMP activates an ATP-permeable pathway in neonatal rat cardiac myocytes.

A. S. Lader, Y.-F. Xiao, C. R. O'Riordan, A. G. Prat, G. R. Jackson Jr., and H. F. Cantiello (2000)
Am J Physiol Cell Physiol 279, C173-C187
 |  Abstract »  |  Full Text »  |  PDF »

Murine colonic mucosa hyperproliferation. I. Elevated CFTR expression and enhanced cAMP-dependent Cl- secretion.

S. Umar, J. Scott, J. H. Sellin, W. P. Dubinsky, and A. P. Morris (2000)
Am J Physiol Gastrointest Liver Physiol 278, G753-G764
 |  Abstract »  |  Full Text »  |  PDF »

The Two Halves of CFTR Form a Dual-pore Ion Channel.

H. Yue, S. Devidas, and W. B. Guggino (2000)
J. Biol. Chem. 275, 10030-10034
 |  Abstract »  |  Full Text »  |  PDF »

A Naturally Occurring Sequence Variation That Creates a YY1 Element Is Associated with Increased Cystic Fibrosis Transmembrane Conductance Regulator Gene Expression.

M.-C. Romey, N. Pallares-Ruiz, A. Mange, C. Mettling, R. Peytavi, J. Demaille, and M. Claustres (2000)
J. Biol. Chem. 275, 3561-3567
 |  Abstract »  |  Full Text »  |  PDF »

The Carboxyl Terminus of the Cystic Fibrosis Transmembrane Conductance Regulator Binds to AP-2 Clathrin Adaptors.

K. M. Weixel and N. A. Bradbury (2000)
J. Biol. Chem. 275, 3655-3660
 |  Abstract »  |  Full Text »  |  PDF »

Anion Transport in Heart.

J. R. Hume, D. Duan, M. L. Collier, J. Yamazaki, and B. Horowitz (2000)
Physiol Rev 80, 31-81
 |  Abstract »  |  Full Text »  |  PDF »

Actin filament organization is required for proper cAMP-dependent activation of CFTR.

A. G. Prat, C. C. Cunningham, G. R. Jackson Jr., S. C. Borkan, Y. Wang, D. A. Ausiello, and H. F. Cantiello (1999)
Am J Physiol Cell Physiol 277, C1160-C1169
 |  Abstract »  |  Full Text »  |  PDF »

Fluid absorption related to ion transport in human airway epithelial spheroids.

P. S. Pedersen, N.-H. Holstein-Rathlou, P. L. Larsen, K. Qvortrup, and O. Frederiksen (1999)
Am J Physiol Lung Cell Mol Physiol 277, L1096-L1103
 |  Abstract »  |  Full Text »  |  PDF »

Intracellular Cl regulates Na-K-Cl cotransport activity in human trabecular meshwork cells.

L. K. Putney, C. R. T. Vibat, and M. E. O'Donnell (1999)
Am J Physiol Cell Physiol 277, C373-C383
 |  Abstract »  |  Full Text »  |  PDF »

Cystic Fibrosis Transmembrane Conductance Regulator Regulates Luminal Cl-/HCO3- Exchange in Mouse Submandibular and Pancreatic Ducts.

M. G. Lee, J. Y. Choi, X. Luo, E. Strickland, P. J. Thomas, and S. Muallem (1999)
J. Biol. Chem. 274, 14670-14677
 |  Abstract »  |  Full Text »  |  PDF »

Characterization of the internalization pathways for the cystic fibrosis transmembrane conductance regulator.

N. A. Bradbury, J. A. Clark, S. C. Watkins, C. C. Widnell, H. S. Smith IV, and R. J. Bridges (1999)
Am J Physiol Lung Cell Mol Physiol 276, L659-L668
 |  Abstract »  |  Full Text »  |  PDF »

Nitric oxide-mediated regulation of transepithelial sodium and chloride transport in murine nasal epithelium.

H. L. Elmer, K. G. Brady, M. L. Drumm, and T. J. Kelley (1999)
Am J Physiol Lung Cell Mol Physiol 276, L466-L473
 |  Abstract »  |  Full Text »  |  PDF »

Regulation of Cl-/ HCO3- Exchange by Cystic Fibrosis Transmembrane Conductance Regulator Expressed in NIH 3T3 and HEK 293 Cells.

M. G. Lee, W. C. Wigley, W. Zeng, L. E. Noel, C. R. Marino, P. J. Thomas, and S. Muallem (1999)
J. Biol. Chem. 274, 3414-3421
 |  Abstract »  |  Full Text »  |  PDF »

Efficient Endocytosis of the Cystic Fibrosis Transmembrane Conductance Regulator Requires a Tyrosine-based Signal.

L. S. Prince, K. Peter, S. R. Hatton, L. Zaliauskiene, L. F. Cotlin, J. P. Clancy, R. B. Marchase, and J. F. Collawn (1999)
J. Biol. Chem. 274, 3602-3609
 |  Abstract »  |  Full Text »  |  PDF »

Cloning, characterization, and functional expression of a CNP receptor regulating CFTR in the shark rectal gland.

S. G. Aller, I. D. Lombardo, S. Bhanot, and J. N. Forrest Jr. (1999)
Am J Physiol Cell Physiol 276, C442-C449
 |  Abstract »  |  Full Text »  |  PDF »

CFTR channel insertion to the apical surface in rat duodenal villus epithelial cells is upregulated by VIP in vivo.

N. Ameen, B Martensson, L Bourguinon, C Marino, J Isenberg, and G. McLaughlin (1999)
J. Cell Sci. 112, 887-894
 |  Abstract »  |  PDF »

A novel plant-derived inhibitor of cAMP-mediated fluid and chloride secretion.

S. E. Gabriel, S. E. Davenport, R. J. Steagall, V. Vimal, T. Carlson, and E. J. Rozhon (1999)
Am J Physiol Gastrointest Liver Physiol 276, G58-G63
 |  Abstract »  |  Full Text »  |  PDF »

Structure and Function of the CFTR Chloride Channel.

D. N. SHEPPARD and M. J. WELSH (1999)
Physiol Rev 79, 23-45
 |  Abstract »  |  Full Text »  |  PDF »

Ion transport in epithelial spheroids derived from human airway cells.

P. S. Pedersen, O. Frederiksen, N.-H. Holstein-Rathlou, P. L. Larsen, and K. Qvortrup (1999)
Am J Physiol Lung Cell Mol Physiol 276, L75-L80
 |  Abstract »  |  Full Text »  |  PDF »

Pharmacology of CFTR Chloride Channel Activity.

B. D. SCHULTZ, A. K. SINGH, D. C. DEVOR, and R. J. BRIDGES (1999)
Physiol Rev 79, 109-144
 |  Abstract »  |  Full Text »  |  PDF »

Intracellular CFTR: Localization and Function.

N. A. BRADBURY (1999)
Physiol Rev 79, 175-191
 |  Abstract »  |  Full Text »  |  PDF »

Pathophysiology of Gene-Targeted Mouse Models for Cystic Fibrosis.

B. R. GRUBB and R. C. BOUCHER (1999)
Physiol Rev 79, 193-214
 |  Abstract »  |  Full Text »  |  PDF »

The Second Half of the Cystic Fibrosis Transmembrane Conductance Regulator Forms a Functional Chloride Channel.

S. Devidas, H. Yue, and W. B. Guggino (1998)
J. Biol. Chem. 273, 29373-29380
 |  Abstract »  |  Full Text »  |  PDF »

Membrane Trafficking of the Cystic Fibrosis Gene Product, Cystic Fibrosis Transmembrane Conductance Regulator, Tagged with Green Fluorescent Protein in Madin-Darby Canine Kidney Cells.

B. D. Moyer, J. Loffing, E. M. Schwiebert, D. Loffing-Cueni, P. A. Halpin, K. H. Karlson, I. I. Ismailov, W. B. Guggino, G. M. Langford, and B. A. Stanton (1998)
J. Biol. Chem. 273, 21759-21768
 |  Abstract »  |  Full Text »  |  PDF »

Effects of Topically Delivered Benzamil and Amiloride on Nasal Potential Difference in Cystic Fibrosis.

T. HOFMANN, M. J. STUTTS, A. ZIERSCH, C. RUCKES, W. M. WEBER, M. R. KNOWLES, H. LINDEMANN, and R. C. BOUCHER (1998)
Am. J. Respir. Crit. Care Med. 157, 1844-1849
 |  Abstract »  |  Full Text »  |  PDF »

Incidence and Prevalence of Neutralizing Antibodies to the Common Adenoviruses in Children With Cystic Fibrosis: Implication for Gene Therapy With Adenovirus Vectors.

P. A. Piedra, G. A. Poveda, B. Ramsey, K. McCoy, and P. W. Hiatt (1998)
Pediatrics 101, 1013-1019
 |  Abstract »  |  Full Text »  |  PDF »

Sertoli cell expression of the cystic fibrosis transmembrane conductance regulator.

F. R. Boockfor, R. A. Morris, D. C. DeSimone, D. M. Hunt, and K. B. Walsh (1998)
Am J Physiol Cell Physiol 274, C922-C930
 |  Abstract »  |  Full Text »  |  PDF »

Direct Activation of Cystic Fibrosis Transmembrane Conductance Regulator Channels by 8-Cyclopentyl-1,3-dipropylxanthine (CPX) and 1,3-Diallyl-8-cyclohexylxanthine (DAX).

N. Arispe, J. Ma, K. A. Jacobson, and H. B. Pollard (1998)
J. Biol. Chem. 273, 5727-5734
 |  Abstract »  |  Full Text »  |  PDF »

Arylaminobenzoate Block of the Cardiac Cyclic AMP-Dependent Chloride Current.

K. B. Walsh and C. Wang (1998)
Mol. Pharmacol. 53, 539-546
 |  Abstract »  |  Full Text »

Inhibition of airway liquid secretion and its effect on the physical properties of airway mucus.

L. Trout, M. King, W. Feng, S. K. Inglis, and S. T. Ballard (1998)
Am J Physiol Lung Cell Mol Physiol 274, L258-L263
 |  Abstract »  |  Full Text »  |  PDF »

Cystic Fibrosis Phenotype Associated with Pancreatic Insufficiency Does Not Always Reflect the cAMP-dependent Chloride Conductive Pathway Defect. ANALYSIS OF C225R-CFTR AND R1066C-CFTR.

P. Fanen, R. Labarthe, F. Garnier, M. Benharouga, M. Goossens, and A. Edelman (1997)
J. Biol. Chem. 272, 30563-30566
 |  Abstract »  |  Full Text »  |  PDF »

Cystic Fibrosis Transmembrane Conductance Regulator-associated ATP and Adenosine 3'-Phosphate 5'-Phosphosulfate Channels in Endoplasmic Reticulum and Plasma Membranes.

E. A. Pasyk and J. K. Foskett (1997)
J. Biol. Chem. 272, 7746-7751
 |  Abstract »  |  Full Text »  |  PDF »

Distribution of cAMP-Activated Chloride Current and CFTR mRNA in the Guinea Pig Heart.

A. F. James, T. Tominaga, Y. Okada, and M. Tominaga (1996)
Circ. Res. 79, 201-207
 |  Abstract »  |  Full Text »

Evidence against Defective trans-Golgi Acidification in Cystic Fibrosis.

O. Seksek, J. Biwersi, and A.S. Verkman (1996)
J. Biol. Chem. 271, 15542-15548
 |  Abstract »  |  Full Text »  |  PDF »

Role of Multidrug Resistance P-glycoproteins in Cholesterol Biosynthesis.

J. E. Metherall, H. Li, and K. Waugh (1996)
J. Biol. Chem. 271, 2634-2640
 |  Abstract »  |  Full Text »  |  PDF »

Glibenclamide, an ATP-Sensitive K+ Channel Blocker, Inhibits Cardiac cAMP-Activated Cl- Conductance.

M. Tominaga, M. Horie, S. Sasayama, and Y. Okada (1995)
Circ. Res. 77, 417-423
 |  Abstract »  |  Full Text »

Alternate Translation Initiation Codons Can Create Functional Forms of Cystic Fibrosis Transmembrane Conductance Regulator.

T. P. Carroll, M. M. Morales, S. B. Fulmer, S. S. Allen, T. R. Flotte, G. R. Cutting, and W. B. Guggino (1995)
J. Biol. Chem. 270, 11941-11946
 |  Abstract »  |  Full Text »  |  PDF »

The Two Nucleotide-binding Domains of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Have Distinct Functions in Controlling Channel Activity.

M. R. Carson, S. M. Travis, and M. J. Welsh (1995)
J. Biol. Chem. 270, 1711-1717
 |  Abstract »  |  Full Text »  |  PDF »

Production and characterisation of monoclonal and polyclonal antibodies to different regions of the cystic fibrosis transmembrane conductance regulator (CFTR): detection of immunologically related proteins.

J Walker, J Watson, C Holmes, A Edelman, and G Banting (1995)
J. Cell Sci. 108, 2433-2444
 |  Abstract »  |  PDF »

Correlation between Genotype and Phenotype in Patients with Cystic Fibrosis.

The Cystic Fibrosis Genotype-Phenotype Consortium (1993)
N. Engl. J. Med. 329, 1308-1313
 |  Abstract »  |  Full Text »

Intestinal Electrolyte Secretion: History of a Paradigm.

M. Field (1993)
Arch Surg 128, 273-278
 |  Abstract »  |  PDF »

Genetics and Physiology of the Myotonic Muscle Disorders.

L. J. Ptacek, K. J. Johnson, and R. C. Griggs (1993)
N. Engl. J. Med. 328, 482-489
 |  Full Text »

A multifunctional aqueous channel formed by CFTR.

H Hasegawa, W Skach, O Baker, M. Calayag, V Lingappa, and A. Verkman (1992)
Science 258, 1477-1479
 |  Abstract »  |  PDF »

Regulation by ATP and ADP of CFTR chloride channels that contain mutant nucleotide-binding domains.

M. Anderson and M. Welsh (1992)
Science 257, 1701-1704
 |  Abstract »  |  PDF »

Cystic fibrosis: molecular biology and therapeutic implications.

F. Collins (1992)
Science 256, 774-779
 |  Abstract »  |  PDF »

Regulation of plasma membrane recycling by CFTR.

N. Bradbury, T Jilling, G Berta, E. Sorscher, R. Bridges, and K. Kirk (1992)
Science 256, 530-532
 |  Abstract »  |  PDF »

Recombinant Human DNase Inhalation in Normal Subjects and Patients With Cystic Fibrosis: A Phase 1 Study.

M. L. Aitken, W. Burke, G. McDonald, S. Shak, A. B. Montgomery, and A. Smith (1992)
JAMA 267, 1947-1951
 |  Abstract »  |  PDF »

Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.

M. Drumm, D. Wilkinson, L. Smit, R. Worrell, T. Strong, R. Frizzell, D. Dawson, and F. Collins (1991)
Science 254, 1797-1799
 |  Abstract »  |  PDF »

Demonstration that CFTR is a chloride channel by alteration of its anion selectivity.

M. Anderson, R. Gregory, S Thompson, D. Souza, S Paul, R. Mulligan, A. Smith, and M. Welsh (1991)
Science 253, 202-205
 |  Abstract »  |  PDF »

Effect of deleting the R domain on CFTR-generated chloride channels.

D. Rich, R. Gregory, M. Anderson, P Manavalan, A. Smith, and M. Welsh (1991)
Science 253, 205-207
 |  Abstract »  |  PDF »

Cystic Fibrosis Transmembrane Conductance Regulator Facilitates ATP Release by Stimulating a Separate ATP Release Channel for Autocrine Control of Cell Volume Regulation.

G. M. Braunstein, R. M. Roman, J. P. Clancy, B. A. Kudlow, A. L. Taylor, V. Gh. Shylonsky, B. Jovov, K. Peter, T. Jilling, I. I. Ismailov, et al. (2001)
J. Biol. Chem. 276, 6621-6630
 |  Abstract »  |  Full Text »  |  PDF »

Post-translational Disruption of the Delta F508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Molecular Chaperone Complex with Geldanamycin Stabilizes Delta F508 CFTR in the Rabbit Reticulocyte Lysate.

W. Fuller and A. W. Cuthbert (2000)
J. Biol. Chem. 275, 37462-37468
 |  Abstract »  |  Full Text »  |  PDF »

Perturbation of the Pore of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Inhibits Its ATPase Activity.

I. Kogan, M. Ramjeesingh, L.-J. Huan, Y. Wang, and C. E. Bear (2001)
J. Biol. Chem. 276, 11575-11581
 |  Abstract »  |  Full Text »  |  PDF »

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