Science 11 August 1995: Vol. 269. no. 5225, pp. 847 - 850 DOI: 10.1126/science.7543698

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Articles

Transmembrane Protein 16A (TMEM16A) Is a Ca2+-regulated Cl- Secretory Channel in Mouse Airways.

J. R. Rock, W. K. O'Neal, S. E. Gabriel, S. H. Randell, B. D. Harfe, R. C. Boucher, and B. R. Grubb (2009)
J. Biol. Chem. 284, 14875-14880
 |  Abstract »  |  Full Text »  |  PDF »

Genetic Analysis of Rwandan Patients With Cystic Fibrosis-Like Symptoms: Identification of Novel Cystic Fibrosis Transmembrane Conductance Regulator and Epithelial Sodium Channel Gene Variants.

L. Mutesa, A. K. Azad, C. Verhaeghe, K. Segers, J.-F. Vanbellinghen, L. Ngendahayo, E. K. Rusingiza, P. R. Mutwa, S. Rulisa, L. Koulischer, et al. (2009)
Chest 135, 1233-1242
 |  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 »

Role of the Cystic Fibrosis Transmembrane Conductance Channel in Human Airway Smooth Muscle.

M.-C. Michoud, R. Robert, M. Hassan, B. Moynihan, C. Haston, V. Govindaraju, P. Ferraro, J. W. Hanrahan, and J. G. Martin (2009)
Am. J. Respir. Cell Mol. Biol. 40, 217-222
 |  Abstract »  |  Full Text »  |  PDF »

Role of the amiloride-sensitive epithelial Na+ channel in the pathogenesis and as a therapeutic target for cystic fibrosis lung disease.

M. A. Mall (2009)
Exp Physiol 94, 171-174
 |  Abstract »  |  Full Text »  |  PDF »

Preventive but Not Late Amiloride Therapy Reduces Morbidity and Mortality of Lung Disease in {beta}ENaC-overexpressing Mice.

Z. Zhou, D. Treis, S. C. Schubert, M. Harm, J. Schatterny, S. Hirtz, J. Duerr, R. C. Boucher, and M. A. Mall (2008)
Am. J. Respir. Crit. Care Med. 178, 1245-1256
 |  Abstract »  |  Full Text »  |  PDF »

Raising the volume on near-silent epithelial Na+ channels.

R. Tarran (2008)
J. Physiol. 586, 4583-4584
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Chemical Rescue of {Delta}F508-CFTR Mimics Genetic Repair in Cystic Fibrosis Bronchial Epithelial Cells.

O. V. Singh, H. B. Pollard, and P. L. Zeitlin (2008)
Mol. Cell. Proteomics 7, 1099-1110
 |  Abstract »  |  Full Text »  |  PDF »

Parallel Improvement of Sodium and Chloride Transport Defects by Miglustat (n-Butyldeoxynojyrimicin) in Cystic Fibrosis Epithelial Cells.

S. Noel, M. Wilke, A. G. M. Bot, H. R. De Jonge, and F. Becq (2008)
J. Pharmacol. Exp. Ther. 325, 1016-1023
 |  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 »

Development of Chronic Bronchitis and Emphysema in {beta}-Epithelial Na+ Channel-Overexpressing Mice.

M. A. Mall, J. R. Harkema, J. B. Trojanek, D. Treis, A. Livraghi, S. Schubert, Z. Zhou, S. M. Kreda, S. L. Tilley, E. J. Hudson, et al. (2008)
Am. J. Respir. Crit. Care Med. 177, 730-742
 |  Abstract »  |  Full Text »  |  PDF »

Purinergic receptors in gastrointestinal inflammation.

V. L. Kolachala, R. Bajaj, M. Chalasani, and S. V. Sitaraman (2008)
Am J Physiol Gastrointest Liver Physiol 294, G401-G410
 |  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 »

Modulation of Epithelial Sodium Channel Trafficking and Function by Sodium 4-Phenylbutyrate in Human Nasal Epithelial Cells.

V. Pruliere-Escabasse, C. Planes, E. Escudier, P. Fanen, A. Coste, and C. Clerici (2007)
J. Biol. Chem. 282, 34048-34057
 |  Abstract »  |  Full Text »  |  PDF »

Sodium Channels and Cystic Fibrosis.

S. H. Donaldson and R. C. Boucher (2007)
Chest 132, 1631-1636
 |  Abstract »  |  Full Text »  |  PDF »

Reproducibility of Nasal Potential Difference Measurements in Cystic Fibrosis.

Y. Yaakov, E. Kerem, Y. Yahav, J. Rivlin, H. Blau, L. Bentur, M. Aviram, E. Picard, T. Bdolah-Abram, and M. Wilschanski (2007)
Chest 132, 1219-1226
 |  Abstract »  |  Full Text »  |  PDF »

WNK4-mediated regulation of renal ion transport proteins.

J.-B. Peng and D. G. Warnock (2007)
Am J Physiol Renal Physiol 293, F961-F973
 |  Abstract »  |  Full Text »  |  PDF »

Pharmacological modulation of cGMP levels by phosphodiesterase 5 inhibitors as a therapeutic strategy for treatment of respiratory pathology in cystic fibrosis.

J. F. Poschet, G. S. Timmins, J. L. Taylor-Cousar, W. Ornatowski, J. Fazio, E. Perkett, K. R. Wilson, H. D. Yu, H. R. de Jonge, and V. Deretic (2007)
Am J Physiol Lung Cell Mol Physiol 293, L712-L719
 |  Abstract »  |  Full Text »  |  PDF »

Acute Downregulation of ENaC by EGF Involves the PY Motif and Putative ERK Phosphorylation Site.

R. A. Falin and C. U. Cotton (2007)
J. Gen. Physiol. 130, 313-328
 |  Abstract »  |  Full Text »  |  PDF »

Olfactory epithelia exhibit progressive functional and morphological defects in CF mice.

B. R. Grubb, T. D. Rogers, H. M. Kulaga, K. A. Burns, R. L. Wonsetler, R. R. Reed, and L. E. Ostrowski (2007)
Am J Physiol Cell Physiol 293, C574-C583
 |  Abstract »  |  Full Text »  |  PDF »

The impact of sex and sex hormones on lung physiology and disease: lessons from animal studies.

M. A. Carey, J. W. Card, J. W. Voltz, D. R. Germolec, K. S. Korach, and D. C. Zeldin (2007)
Am J Physiol Lung Cell Mol Physiol 293, L272-L278
 |  Abstract »  |  Full Text »  |  PDF »

Cystic Fibrosis: Lessons from the Sweat Gland.

P. M. Quinton (2007)
Physiology 22, 212-225
 |  Abstract »  |  Full Text »  |  PDF »

Protein Kinase CK2, Cystic Fibrosis Transmembrane Conductance Regulator, and the {Delta}F508 Mutation: F508 DELETION DISRUPTS A KINASE-BINDING SITE.

K. J. Treharne, R. M. Crawford, Z. Xu, J.-H. Chen, O. G. Best, E. A. Schulte, D. C. Gruenert, S. M. Wilson, D. N. Sheppard, K. Kunzelmann, et al. (2007)
J. Biol. Chem. 282, 10804-10813
 |  Abstract »  |  Full Text »  |  PDF »

CFTR inhibition mimics the cystic fibrosis inflammatory profile.

A. Perez, A. C. Issler, C. U. Cotton, T. J. Kelley, A. S. Verkman, and P. B. Davis (2007)
Am J Physiol Lung Cell Mol Physiol 292, L383-L395
 |  Abstract »  |  Full Text »  |  PDF »

Cystic Fibrosis Mouse Models.

C. Guilbault, Z. Saeed, G. P. Downey, and D. Radzioch (2007)
Am. J. Respir. Cell Mol. Biol. 36, 1-7
 |  Abstract »  |  Full Text »  |  PDF »

An Extract from the Medicinal Plant Phyllanthus acidus and Its Isolated Compounds Induce Airway Chloride Secretion: A Potential Treatment for Cystic Fibrosis.

M. Sousa, J. Ousingsawat, R. Seitz, S. Puntheeranurak, A. Regalado, A. Schmidt, T. Grego, C. Jansakul, M. D. Amaral, R. Schreiber, et al. (2007)
Mol. Pharmacol. 71, 366-376
 |  Abstract »  |  Full Text »  |  PDF »

Acinar origin of CFTR-dependent airway submucosal gland fluid secretion.

J. V. Wu, M. E. Krouse, and J. J. Wine (2007)
Am J Physiol Lung Cell Mol Physiol 292, L304-L311
 |  Abstract »  |  Full Text »  |  PDF »

Absence of CFTR is associated with pleiotropic effects on mucins in mouse gallbladder epithelial cells.

R. Kuver, T. Wong, J. H. Klinkspoor, and S. P. Lee (2006)
Am J Physiol Gastrointest Liver Physiol 291, G1148-G1154
 |  Abstract »  |  Full Text »  |  PDF »

Oligomerization of KCC2 Correlates with Development of Inhibitory Neurotransmission.

P. Blaesse, I. Guillemin, J. Schindler, M. Schweizer, E. Delpire, L. Khiroug, E. Friauf, and H. G. Nothwang (2006)
J. Neurosci. 26, 10407-10419
 |  Abstract »  |  Full Text »  |  PDF »

Mutations in the Cystic Fibrosis Transmembrane Regulator Gene and In Vivo Transepithelial Potentials.

M. Wilschanski, A. Dupuis, L. Ellis, K. Jarvi, J. Zielenski, E. Tullis, S. Martin, M. Corey, L.-C. Tsui, and P. Durie (2006)
Am. J. Respir. Crit. Care Med. 174, 787-794
 |  Abstract »  |  Full Text »  |  PDF »

Airway Surface Liquid Volume Regulates ENaC by Altering the Serine Protease-Protease Inhibitor Balance: A MECHANISM FOR SODIUM HYPERABSORPTION IN CYSTIC FIBROSIS.

M. M. Myerburg, M. B. Butterworth, E. E. McKenna, K. W. Peters, R. A. Frizzell, T. R. Kleyman, and J. M. Pilewski (2006)
J. Biol. Chem. 281, 27942-27949
 |  Abstract »  |  Full Text »  |  PDF »

Alveolar Epithelial Ion and Fluid Transport: Recent Progress.

H. G. Folkesson and M. A. Matthay (2006)
Am. J. Respir. Cell Mol. Biol. 35, 10-19
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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
 |  Abstract »  |  Full Text »  |  PDF »

PVD9902, a porcine vas deferens epithelial cell line that exhibits neurotransmitter-stimulated anion secretion and expresses numerous HCO3- transporters.

R. W. Carlin, R. L. Sedlacek, R. R. Quesnell, F. Pierucci-Alves, D. M. Grieger, and B. D. Schultz (2006)
Am J Physiol Cell Physiol 290, C1560-C1571
 |  Abstract »  |  Full Text »  |  PDF »

Soluble Mediators, Not Cilia, Determine Airway Surface Liquid Volume in Normal and Cystic Fibrosis Superficial Airway Epithelia.

R. Tarran, L. Trout, S. H. Donaldson, and R. C. Boucher (2006)
J. Gen. Physiol. 127, 591-604
 |  Abstract »  |  Full Text »  |  PDF »

Differential effects of Hsc70 and Hsp70 on the intracellular trafficking and functional expression of epithelial sodium channels.

S. B. Goldfarb, O. B. Kashlan, J. N. Watkins, L. Suaud, W. Yan, T. R. Kleyman, and R. C. Rubenstein (2006)
PNAS 103, 5817-5822
 |  Abstract »  |  Full Text »  |  PDF »

Mechanisms of Cystic Fibrosis Transmembrane Conductance Regulator Activation by S-Nitrosoglutathione.

L. Chen, R. P. Patel, X. Teng, C. A. Bosworth, J. R. Lancaster Jr., and S. Matalon (2006)
J. Biol. Chem. 281, 9190-9199
 |  Abstract »  |  Full Text »  |  PDF »

Atrial natriuretic peptide modulates cystic fibrosis transmembrane conductance regulator chloride channel expression in rat proximal colon and human intestinal epithelial cells..

H J Novaira, D S Ornellas, T M Ortiga-Carvalho, X M Zhang, J Souza-Menezes, S E Guggino, W B Guggino, and M M Morales (2006)
J. Endocrinol. 189, 155-165
 |  Abstract »  |  Full Text »  |  PDF »

Talniflumate Increases Survival in a Cystic Fibrosis Mouse Model of Distal Intestinal Obstructive Syndrome.

N. M. Walker, J. E. Simpson, R. C. Levitt, K. T. Boyle, and L. L. Clarke (2006)
J. Pharmacol. Exp. Ther. 317, 275-283
 |  Abstract »  |  Full Text »  |  PDF »

Restoration of Mucociliary Transport in the Fluid-DepletedTrachea by Surface-Active Instillates.

S. T. Ballard, J. C. Parker, and C. R. Hamm (2006)
Am. J. Respir. Cell Mol. Biol. 34, 500-504
 |  Abstract »  |  Full Text »  |  PDF »

Hyposecretion, Not Hyperabsorption, Is the Basic Defect of Cystic Fibrosis Airway Glands.

N. S. Joo, T. Irokawa, R. C. Robbins, and J. J. Wine (2006)
J. Biol. Chem. 281, 7392-7398
 |  Abstract »  |  Full Text »  |  PDF »

Glucocorticoid regulation of genes in the amiloride-sensitive sodium transport pathway by semicircular canal duct epithelium of neonatal rat.

S. R. Pondugula, N. N. Raveendran, Z. Ergonul, Y. Deng, J. Chen, J. D. Sanneman, L. G. Palmer, and D. C. Marcus (2006)
Physiol Genomics 24, 114-123
 |  Abstract »  |  Full Text »  |  PDF »

Regulation of ROMK (Kir1.1) channels: new mechanisms and aspects.

W.-H. Wang (2006)
Am J Physiol Renal Physiol 290, F14-F19
 |  Abstract »  |  Full Text »  |  PDF »

CFTR, chloride concentration and cell volume: could mammalian protein histidine phosphorylation play a latent role?.

K. J Treharne, R. M Crawford, and A Mehta (2006)
Exp Physiol 91, 131-139
 |  Abstract »  |  Full Text »  |  PDF »

Phosphatidylinositol 3,4,5-Trisphosphate Mediates Aldosterone Stimulation of Epithelial Sodium Channel (ENaC) and Interacts with {gamma}-ENaC.

M. N. Helms, L. Liu, Y.-Y. Liang, O. Al-Khalili, A. Vandewalle, S. Saxena, D. C. Eaton, and H.-P. Ma (2005)
J. Biol. Chem. 280, 40885-40891
 |  Abstract »  |  Full Text »  |  PDF »

Estrogen-Induced Abnormally High Cystic Fibrosis Transmembrane Conductance Regulator Expression Results in Ovarian Hyperstimulation Syndrome.

L. C. Ajonuma, L. L. Tsang, G. H. Zhang, C. H. Y. Wong, M. C. Lau, L. S. Ho, D. K. Rowlands, C. X. Zhou, C. P. Ng, J. Chen, et al. (2005)
Mol. Endocrinol. 19, 3038-3044
 |  Abstract »  |  Full Text »  |  PDF »

Mutations in the beta-subunit of the epithelial Na+ channel in patients with a cystic fibrosis-like syndrome.

M. B. Sheridan, P. Fong, J. D. Groman, C. Conrad, P. Flume, R. Diaz, C. Harris, M. Knowles, and G. R. Cutting (2005)
Hum. Mol. Genet. 14, 3493-3498
 |  Abstract »  |  Full Text »  |  PDF »

Normal and Cystic Fibrosis Airway Surface Liquid Homeostasis: THE EFFECTS OF PHASIC SHEAR STRESS AND VIRAL INFECTIONS.

R. Tarran, B. Button, M. Picher, A. M. Paradiso, C. M. Ribeiro, E. R. Lazarowski, L. Zhang, P. L. Collins, R. J. Pickles, J. J. Fredberg, et al. (2005)
J. Biol. Chem. 280, 35751-35759
 |  Abstract »  |  Full Text »  |  PDF »

Alveolar Epithelium: Role in Lung Fluid Balance and Acute Lung Injury.

M. A. Matthay, L. Robriquet, and X. Fang (2005)
Proceedings of the ATS 2, 206-213
 |  Abstract »  |  Full Text »  |  PDF »

Down-regulation of the Anti-inflammatory Protein Annexin A1 in Cystic Fibrosis Knock-out Mice and Patients.

N. Bensalem, A. P. Ventura, B. Vallee, J. Lipecka, D. Tondelier, N. Davezac, A. D. Santos, M. Perretti, A. Fajac, I. Sermet-Gaudelus, et al. (2005)
Mol. Cell. Proteomics 4, 1591-1601
 |  Abstract »  |  Full Text »  |  PDF »

Cl- Interference with the Epithelial Na+ Channel ENaC.

T. Bachhuber, J. Konig, T. Voelcker, B. Murle, R. Schreiber, and K. Kunzelmann (2005)
J. Biol. Chem. 280, 31587-31594
 |  Abstract »  |  Full Text »  |  PDF »

Inositol polyphosphate derivative inhibits Na+ transport and improves fluid dynamics in cystic fibrosis airway epithelia.

M. Moody, C. Pennington, C. Schultz, R. Caldwell, C. Dinkel, M. W. Rossi, S. McNamara, J. Widdicombe, S. Gabriel, and A. E. Traynor-Kaplan (2005)
Am J Physiol Cell Physiol 289, C512-C520
 |  Abstract »  |  Full Text »  |  PDF »

Structure and Function of CLCA Proteins.

M. E. Loewen and G. W. Forsyth (2005)
Physiol Rev 85, 1061-1092
 |  Abstract »  |  Full Text »  |  PDF »

Platelet activation in cystic fibrosis.

B. P. O'Sullivan, M. D. Linden, A. L. Frelinger III, M. R. Barnard, M. Spencer-Manzon, J. E. Morris, R. O. Salem, M. Laposata, and A. D. Michelson (2005)
Blood 105, 4635-4641
 |  Abstract »  |  Full Text »  |  PDF »

Interdependency of {beta}-Adrenergic Receptors and CFTR in Regulation of Alveolar Active Na+ Transport.

G. M. Mutlu, Y. Adir, M. Jameel, A. T. Akhmedov, L. Welch, V. Dumasius, F. J. Meng, J. Zabner, C. Koenig, E. R. Lewis, et al. (2005)
Circ. Res. 96, 999-1005
 |  Abstract »  |  Full Text »  |  PDF »

Epithelial Sodium Channel Inhibition by AMP-activated Protein Kinase in Oocytes and Polarized Renal Epithelial Cells.

M. D. Carattino, R. S. Edinger, H. J. Grieser, R. Wise, D. Neumann, U. Schlattner, J. P. Johnson, T. R. Kleyman, and K. R. Hallows (2005)
J. Biol. Chem. 280, 17608-17616
 |  Abstract »  |  Full Text »  |  PDF »

Increased cystic fibrosis transmembrane conductance regulator (CFTR) expression in the human hydrosalpinx.

L. C. Ajonuma, E. H. Y. Ng, P. H. Chow, C. Y. Hung, L. L. Tsang, A. N. Y. Cheung, C. Brito-Jones, I. H. Lok, C. J.Haines, and H. C. Chan (2005)
Hum. Reprod. 20, 1228-1234
 |  Abstract »  |  Full Text »  |  PDF »

CFTR fails to inhibit the epithelial sodium channel ENaC expressed in Xenopus laevis oocytes.

G. Nagel, P. Barbry, H. Chabot, E. Brochiero, K. Hartung, and R. Grygorczyk (2005)
J. Physiol. 564, 671-682
 |  Abstract »  |  Full Text »  |  PDF »

Neutrophil elastase activates near-silent epithelial Na+ channels and increases airway epithelial Na+ transport.

R. A. Caldwell, R. C. Boucher, and M. J. Stutts (2005)
Am J Physiol Lung Cell Mol Physiol 288, L813-L819
 |  Abstract »  |  Full Text »  |  PDF »

Vasopressin-stimulated CFTR Cl- currents are increased in the renal collecting duct cells of a mouse model of Liddle's syndrome.

C.-T. Chang, M. Bens, E. Hummler, S. Boulkroun, L. Schild, J. Teulon, B. C. Rossier, and A. Vandewalle (2005)
J. Physiol. 562, 271-284
 |  Abstract »  |  Full Text »  |  PDF »

Evaluation of Second Generation Amiloride Analogs as Therapy for Cystic Fibrosis Lung Disease.

A. J. Hirsh, J. R. Sabater, A. Zamurs, R. T. Smith, A. M. Paradiso, S. Hopkins, W. M. Abraham, and R. C. Boucher (2004)
J. Pharmacol. Exp. Ther. 311, 929-938
 |  Abstract »  |  Full Text »  |  PDF »

Nasal airway ion transport is linked to the cystic fibrosis phenotype in adult patients.

I Fajac, D Hubert, D Guillemot, I Honore, T Bienvenu, F Volter, J Dall'Ava-Santucci, and D J Dusser (2004)
Thorax 59, 971-976
 |  Abstract »  |  Full Text »  |  PDF »

Prostasin, a membrane-anchored serine peptidase, regulates sodium currents in JME/CF15 cells, a cystic fibrosis airway epithelial cell line.

Z. Tong, B. Illek, V. J. Bhagwandin, G. M. Verghese, and G. H. Caughey (2004)
Am J Physiol Lung Cell Mol Physiol 287, L928-L935
 |  Abstract »  |  Full Text »  |  PDF »

cAMP and Serum and Glucocorticoid-inducible Kinase (SGK) Regulate the Epithelial Na+ Channel through Convergent Phosphorylation of Nedd4-2.

P. M. Snyder, D. R. Olson, R. Kabra, R. Zhou, and J. C. Steines (2004)
J. Biol. Chem. 279, 45753-45758
 |  Abstract »  |  Full Text »  |  PDF »

AVP-induced VIT32 gene expression in collecting duct cells occurs via trans-activation of a CRE in the 5'-flanking region of the VIT32 gene.

C. P. Thomas, R. W. Loftus, and K. Z. Liu (2004)
Am J Physiol Renal Physiol 287, F460-F468
 |  Abstract »  |  Full Text »  |  PDF »

Activation of Airway Cl- Secretion in Human Subjects by Adenosine.

K. Hentchel-Franks, D. Lozano, V. Eubanks-Tarn, B. Cobb, L. Fan, R. Oster, E. Sorscher, and J. P. Clancy (2004)
Am. J. Respir. Cell Mol. Biol. 31, 140-146
 |  Abstract »  |  Full Text »  |  PDF »

A domain mimic increases {Delta}F508 CFTR trafficking and restores cAMP-stimulated anion secretion in cystic fibrosis epithelia.

L. L. Clarke, L. R. Gawenis, T.-C. Hwang, N. M. Walker, D. B. Gruis, and E. M. Price (2004)
Am J Physiol Cell Physiol 287, C192-C199
 |  Abstract »  |  Full Text »  |  PDF »

Mutations in the extracellular loop of {alpha}-rENaC alter sensitivity to amiloride and reactive species.

L. Chen, C. M. Fuller, T. R. Kleyman, and S. Matalon (2004)
Am J Physiol Renal Physiol 286, F1202-F1208
 |  Abstract »  |  Full Text »  |  PDF »

Additional Disruption of the ClC-2 Cl- Channel Does Not Exacerbate the Cystic Fibrosis Phenotype of Cystic Fibrosis Transmembrane Conductance Regulator Mouse Models.

A. A. Zdebik, J. E. Cuffe, M. Bertog, C. Korbmacher, and T. J. Jentsch (2004)
J. Biol. Chem. 279, 22276-22283
 |  Abstract »  |  Full Text »  |  PDF »

Efficient Intracellular Processing of the Endogenous Cystic Fibrosis Transmembrane Conductance Regulator in Epithelial Cell Lines.

K. Varga, A. Jurkuvenaite, J. Wakefield, J. S. Hong, J. S. Guimbellot, C. J. Venglarik, A. Niraj, M. Mazur, E. J. Sorscher, J. F. Collawn, et al. (2004)
J. Biol. Chem. 279, 22578-22584
 |  Abstract »  |  Full Text »  |  PDF »

Electrophysiological Properties of the Airway: Epithelium in the Murine, Ovalbumin Model of Allergic Airway Disease.

M. M. Cloutier, L. Guernsey, C. A. Wu, and R. S. Thrall (2004)
Am. J. Pathol. 164, 1849-1856
 |  Abstract »  |  Full Text »  |  PDF »

Syntaxin 8 impairs trafficking of cystic fibrosis transmembrane conductance regulator (CFTR) and inhibits its channel activity.

F. Bilan, V. Thoreau, M. Nacfer, R. Derand, C. Norez, A. Cantereau, M. Garcia, F. Becq, and A. Kitzis (2004)
J. Cell Sci. 117, 1923-1935
 |  Abstract »  |  Full Text »  |  PDF »

Impaired Regulatory Volume Decrease in Freshly Isolated Cholangiocytes from Cystic Fibrosis Mice: IMPLICATIONS FOR CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR EFFECT ON POTASSIUM CONDUCTANCE.

W. K. Cho, V. J. Siegrist, and W. Zinzow (2004)
J. Biol. Chem. 279, 14610-14618
 |  Abstract »  |  Full Text »  |  PDF »

Evidence for Up-regulation of the Endogenous Na-K-2Cl Co-transporter by Molecular Interactions with the Anion Exchanger tAE1 Expressed in Xenopus Oocyte.

H. Guizouarn, N. Gabillat, and F. Borgese (2004)
J. Biol. Chem. 279, 11513-11520
 |  Abstract »  |  Full Text »  |  PDF »

Regulation of MUC5AC mucin secretion and airway surface liquid metabolism by IL-1{beta} in human bronchial epithelia.

T. Gray, R. Coakley, A. Hirsh, D. Thornton, S. Kirkham, J.-S. Koo, L. Burch, R. Boucher, and P. Nettesheim (2004)
Am J Physiol Lung Cell Mol Physiol 286, L320-L330
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Modulation of Mature Cystic Fibrosis Transmembrane Regulator Protein by the PDZ Domain Protein CAL.

J. Cheng, H. Wang, and W. B. Guggino (2004)
J. Biol. Chem. 279, 1892-1898
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New concepts of the pathogenesis of cystic fibrosis lung disease.

R.C. Boucher (2004)
Eur. Respir. J. 23, 146-158
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Regulation of Airway Surface Liquid Volume and Mucus Transport by Active Ion Transport.

R. Tarran (2004)
Proceedings of the ATS 1, 42-46
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Serine protease activation of near-silent epithelial Na+ channels.

R. A. Caldwell, R. C. Boucher, and M. J. Stutts (2004)
Am J Physiol Cell Physiol 286, C190-C194
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Airway Surface Liquid Calcium Modulates Chloride Permeability in the Cystic Fibrosis Airway.

P. G. Middleton, K. A. Pollard, E. Donohoo, J. R. Wheatley, D. M. Geddes, and E. W. Alton (2003)
Am. J. Respir. Crit. Care Med. 168, 1223-1226
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Activation of AMP-activated protein kinase reduces cAMP-mediated epithelial chloride secretion.

J. Walker, H. B. Jijon, T. Churchill, M. Kulka, and K. L. Madsen (2003)
Am J Physiol Gastrointest Liver Physiol 285, G850-G860
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Regulation of the Epithelial Na+ Channel by Cytosolic ATP.

T. Ishikawa, C. Jiang, M. J. Stutts, Y. Marunaka, and D. Rotin (2003)
J. Biol. Chem. 278, 38276-38286
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Nasal Airway Ion Transport and Lung Function in Young People with Cystic Fibrosis.

H. L. Wallace, P. M. Barker, and K. W. Southern (2003)
Am. J. Respir. Crit. Care Med. 168, 594-600
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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
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Expression and regulation of the Na+-K+-2Cl- cotransporter NKCC1 in the Normal and CFTR-deficient murine colon.

O Bachmann, K Wuchner, H Rossmann, J Leipziger, B Osikowska, W H Colledge, R Ratcliff, M J Evans, M Gregor, and U Seidler (2003)
J. Physiol. 549, 525-536
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Fluticasone reduces IL-6 and IL-8 production of cystic fibrosis bronchial epithelial cells via IKK-{beta} kinase pathway.

S. Escotte, O. Tabary, D. Dusser, C. Majer-Teboul, E. Puchelle, and J. Jacquot (2003)
Eur. Respir. J. 21, 574-581
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CFTR null mutation altered cAMP-sensitive and swelling-activated Cl- currents in primary cultures of mouse nephron.

H. Barriere, R. Belfodil, I. Rubera, M. Tauc, C. Poujeol, M. Bidet, and P. Poujeol (2003)
Am J Physiol Renal Physiol 284, F796-F811
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Nasal Epithelium Potential Difference at High Altitude (4,559 m): Evidence for Secretion.

H. Mairbaurl, J. Weymann, A. Mohrlein, E. R. Swenson, M. Maggiorini, J. S. R. Gibbs, and P. Bartsch (2003)
Am. J. Respir. Crit. Care Med. 167, 862-867
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Epidermal growth factor inhibits amiloride-sensitive sodium absorption in renal collecting duct cells.

J.-P. Shen and C. U. Cotton (2003)
Am J Physiol Renal Physiol 284, F57-F64
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Multicistronic Lentiviral Vector-Mediated Striatal Gene Transfer of Aromatic L-Amino Acid Decarboxylase, Tyrosine Hydroxylase, and GTP Cyclohydrolase I Induces Sustained Transgene Expression, Dopamine Production, and Functional Improvement in a Rat Model of Parkinson's Disease..

M. Azzouz, E. Martin-Rendon, R. D. Barber, K. A. Mitrophanous, E. E. Carter, J. B. Rohll, S. M. Kingsman, A. J. Kingsman, and N. D. Mazarakis (2002)
J. Neurosci. 22, 10302-10312
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Chloride channels in the kidney: lessons learned from knockout animals.

O. Devuyst and W. B. Guggino (2002)
Am J Physiol Renal Physiol 283, F1176-F1191
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Alveolar epithelial fluid transport in acute lung injury: new insights.

C. Sartori and M.A. Matthay (2002)
Eur. Respir. J. 20, 1299-1313
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Cystic Fibrosis Transmembrane Conductance Regulator Modulates Neurosecretory Function in Pulmonary Neuroendocrine Cell-Related Tumor Cell Line Models.

J. Pan, C. Bear, S. Farragher, E. Cutz, and H. Yeger (2002)
Am. J. Respir. Cell Mol. Biol. 27, 553-560
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Anion channels influence ECC by modulating L-type Ca2+ channel in ventricular myocytes.

S.-S. Zhou, Z. Gao, L. Dong, Y.-F. Ding, X.-D. Zhang, Y.-M. Wang, J.-M. Pei, F. Gao, and X.-L. Ma (2002)
J Appl Physiol 93, 1660-1668
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Reactive Oxygen Nitrogen Species Decrease Cystic Fibrosis Transmembrane Conductance Regulator Expression and cAMP-mediated Cl- Secretion in Airway Epithelia.

Z. Bebok, K. Varga, J. K. Hicks, C. J. Venglarik, T. Kovacs, L. Chen, K. M. Hardiman, J. F. Collawn, E. J. Sorscher, and S. Matalon (2002)
J. Biol. Chem. 277, 43041-43049
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Concerted action of ENaC, Nedd4-2, and Sgk1 in transepithelial Na+ transport.

E. Kamynina and O. Staub (2002)
Am J Physiol Renal Physiol 283, F377-F387
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Regulation of Murine Airway Surface Liquid Volume by CFTR and Ca2+-activated Cl- Conductances.

R. Tarran, M. E. Loewen, A. M. Paradiso, J. C. Olsen, M. A. Gray, B. E. Argent, R. C. Boucher, and S. E. Gabriel (2002)
J. Gen. Physiol. 120, 407-418
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Cystic Fibrosis Transmembrane Conductance Regulator-dependent Up-regulation of Kir1.1 (ROMK) Renal K+ Channels by the Epithelial Sodium Channel.

A.-A. Konstas, J.-P. Koch, S. J. Tucker, and C. Korbmacher (2002)
J. Biol. Chem. 277, 25377-25384
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Lung Epithelial Fluid Transport and the Resolution of Pulmonary Edema.

M. A. Matthay, H. G. Folkesson, and C. Clerici (2002)
Physiol Rev 82, 569-600
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cAMP Increases Density of ENaC Subunits in the Apical Membrane of MDCK Cells in Direct Proportion to Amiloride-sensitive Na+ Transport.

R. G. Morris and J. A. Schafer (2002)
J. Gen. Physiol. 120, 71-85
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