Note to users. If you're seeing this message, it means that your browser cannot find this page's style/presentation instructions -- or possibly that you are using a browser that does not support current Web standards. Find out more about why this message is appearing, and what you can do to make your experience of our site the best it can be.

Science 21 August 1992:
Vol. 257. no. 5073, pp. 1083 - 1088
DOI: 10.1126/science.257.5073.1083
Prev | Table of Contents | Next

Articles

An Animal Model for Cystic Fibrosis Made by Gene Targeting

John N. Snouwaert 1, Kristen K. Brigman 1, Anne M. Latour 1, Nadia N. Malouf 2, Richard C. Boucher 1, Oliver Smithies 2, and Beverly H. Koller 1

1 Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599-7020
2 Department of Pathology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599-7525

Cystic fibrosis results from defects in the gene encoding a cyclic adenosine monophosphate-dependent chloride ion channel known as the cystic fibrosis transmembrane conductance regulator (CFTR). To create an animal model for cystic fibrosis, mice were generated from embryonic stem cells in which the CFTR gene was disrupted by gene targeting. Mice homozygous for the disrupted gene display many features common to young human cystic fibrosis patients, including failure to thrive, meconium ileus, alteration of mucous and serous glands, and obstruction of glandlike structures with inspissated eosinophilic material. Death resulting from intestinal obstruction usually occurs before 40 days of age.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
A guide to Ussing chamber studies of mouse intestine.
L. L. Clarke (2009)
Am J Physiol Gastrointest Liver Physiol 296, G1151-G1166
   Abstract »    Full Text »    PDF »
Reduced NHE3-mediated Na+ absorption increases survival and decreases the incidence of intestinal obstructions in cystic fibrosis mice.
E. M. Bradford, M. A. Sartor, L. R. Gawenis, L. L. Clarke, and G. E. Shull (2009)
Am J Physiol Gastrointest Liver Physiol 296, G886-G898
   Abstract »    Full Text »    PDF »
Poly-L-aspartic Acid Enhances and Prolongs Gentamicin-mediated Suppression of the CFTR-G542X Mutation in a Cystic Fibrosis Mouse Model.
M. Du, K. M. Keeling, L. Fan, X. Liu, and D. M. Bedwell (2009)
J. Biol. Chem. 284, 6885-6892
   Abstract »    Full Text »    PDF »
Macrophages Directly Contribute to the Exaggerated Inflammatory Response in Cystic Fibrosis Transmembrane Conductance Regulator-/- Mice.
E. M. Bruscia, P.-X. Zhang, E. Ferreira, C. Caputo, J. W. Emerson, D. Tuck, D. S. Krause, and M. E. Egan (2009)
Am. J. Respir. Cell Mol. Biol. 40, 295-304
   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 »
Validation of Nasal Potential Difference Measurements in Gut-Corrected CF Knockout Mice.
U. Griesenbach, S. N. Smith, R. Farley, C. Singh, and E. W. F. W. Alton (2008)
Am. J. Respir. Cell Mol. Biol. 39, 490-496
   Abstract »    Full Text »    PDF »
Gender-Specific Protection of Estrogen against Gastric Acid-Induced Duodenal Injury: Stimulation of Duodenal Mucosal Bicarbonate Secretion.
A. Smith, C. Contreras, K. H. Ko, J. Chow, X. Dong, B. Tuo, H.-h. Zhang, D.-b. Chen, and H. Dong (2008)
Endocrinology 149, 4554-4566
   Abstract »    Full Text »    PDF »
A2B Adenosine Receptors Regulate the Mucus Clearance Component of the Lung's Innate Defense System.
B. M. Rollins, M. Burn, R. D. Coakley, L. A. Chambers, A. J. Hirsh, M. T. Clunes, M. I. Lethem, S. H. Donaldson, and R. Tarran (2008)
Am. J. Respir. Cell Mol. Biol. 39, 190-197
   Abstract »    Full Text »    PDF »
Nasal Abnormalities in Cystic Fibrosis Mice Independent of Infection and Inflammation.
T. N. Hilliard, J. Zhu, R. Farley, S. Escudero-Garcia, B. J. Wainwright, P. K. Jeffery, U. Griesenbach, A. Bush, J. C. Davies, and E. W. F. W. Alton (2008)
Am. J. Respir. Cell Mol. Biol. 39, 19-25
   Abstract »    Full Text »    PDF »
Congenital tracheal malformation in cystic fibrosis transmembrane conductance regulator-deficient mice.
E. Bonvin, P. Le Rouzic, J.-F. Bernaudin, C.-H. Cottart, C. Vandebrouck, A. Crie, T. Leal, A. Clement, and M. Bonora (2008)
J. Physiol. 586, 3231-3243
   Abstract »    Full Text »    PDF »
Infertility in Females with Cystic Fibrosis Is Multifactorial: Evidence from Mouse Models.
C. A. Hodges, M. R. Palmert, and M. L. Drumm (2008)
Endocrinology 149, 2790-2797
   Abstract »    Full Text »    PDF »
Heat-stable enterotoxin of Escherichia coli (STa) can stimulate duodenal HCO3- secretion via a novel GC-C- and CFTR-independent pathway.
Z. M. Sellers, E. Mann, A. Smith, K. H. Ko, R. Giannella, M. B. Cohen, K. E. Barrett, and H. Dong (2008)
FASEB J 22, 1306-1316
   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 »
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 »
Persistent Osteopenia in Adult Cystic Fibrosis Transmembrane Conductance Regulator-deficient Mice.
C. K. Haston, W. Li, A. Li, M. Lafleur, and J. E. Henderson (2008)
Am. J. Respir. Crit. Care Med. 177, 309-315
   Abstract »    Full Text »    PDF »
Hormonal control of the renal immune response and antibacterial host defense by arginine vasopressin.
C. Chassin, M. W. Hornef, M. Bens, M. Lotz, J.-M. Goujon, S. Vimont, G. Arlet, A. Hertig, E. Rondeau, and A. Vandewalle (2007)
J. Exp. Med. 204, 2837-2852
   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 »
Abolition of Ca2+-mediated intestinal anion secretion and increased stool dehydration in mice lacking the intermediate conductance Ca2+-dependent K+ channel Kcnn4.
C. A. Flores, J. E. Melvin, C. D. Figueroa, and F. V. Sepulveda (2007)
J. Physiol. 583, 705-717
   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 »
Intestinal phenotype of variable-weight cystic fibrosis knockout mice.
J. C. Canale-Zambrano, M. C. Poffenberger, S. M. Cory, D. G. Humes, and C. K. Haston (2007)
Am J Physiol Gastrointest Liver Physiol 293, G222-G229
   Abstract »    Full Text »    PDF »
Acute inflammation alters bicarbonate transport in mouse ileum.
H. Zhang, N. Ameen, J. E. Melvin, and S. Vidyasagar (2007)
J. Physiol. 581, 1221-1233
   Abstract »    Full Text »    PDF »
Cystic fibrosis transmembrane conductance regulator is vital to sperm fertilizing capacity and male fertility.
W. M. Xu, Q. X. Shi, W. Y. Chen, C. X. Zhou, Y. Ni, D. K. Rowlands, G. Yi Liu, H. Zhu, Z. G. Ma, X. F. Wang, et al. (2007)
PNAS 104, 9816-9821
   Abstract »    Full Text »    PDF »
A role for CFTR in the elevation of glutathione levels in the lung by oral glutathione administration.
C. Kariya, H. Leitner, E. Min, C. van Heeckeren, A. van Heeckeren, and B. J. Day (2007)
Am J Physiol Lung Cell Mol Physiol 292, L1590-L1597
   Abstract »    Full Text »    PDF »
Colonic Anion Secretory Defects and Metabolic Acidosis in Mice Lacking the NBC1 Formula Cotransporter.
L. R. Gawenis, E. M. Bradford, V. Prasad, J. N. Lorenz, J. E. Simpson, L. L. Clarke, A. L. Woo, C. Grisham, L. P. Sanford, T. Doetschman, et al. (2007)
J. Biol. Chem. 282, 9042-9052
   Abstract »    Full Text »    PDF »
Long-term docosahexaenoic acid therapy in a congenic murine model of cystic fibrosis.
S. Beharry, C. Ackerley, M. Corey, G. Kent, Y.-M. Heng, H. Christensen, C. Luk, R. K. Yantiss, I. A. Nasser, M. Zaman, et al. (2007)
Am J Physiol Gastrointest Liver Physiol 292, G839-G848
   Abstract »    Full Text »    PDF »
Extracellular pH alkalinization by Cl-/HCO3- exchanger is crucial for TASK2 activation by hypotonic shock in proximal cell lines from mouse kidney.
S. L'Hoste, H. Barriere, R. Belfodil, I. Rubera, C. Duranton, M. Tauc, C. Poujeol, J. Barhanin, and P. Poujeol (2007)
Am J Physiol Renal Physiol 292, F628-F638
   Abstract »    Full Text »    PDF »
Cystic Fibrosis and Other Respiratory Diseases of Impaired Mucus Clearance.
A. Livraghi and S. H. Randell (2007)
Toxicol Pathol 35, 116-129
   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 »
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 »
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
   Abstract »    Full Text »    PDF »
Increased levels of mucins in the cystic fibrosis mouse small intestine, and modulator effects of the Muc1 mucin expression.
E. K. Malmberg, K. A. Noaksson, M. Phillipson, M. E. V. Johansson, M. Hinojosa-Kurtzberg, L. Holm, S. J. Gendler, and G. C. Hansson (2006)
Am J Physiol Gastrointest Liver Physiol 291, G203-G210
   Abstract »    Full Text »    PDF »
FAT10/Diubiquitin-Like Protein-Deficient Mice Exhibit Minimal Phenotypic Differences..
A. Canaan, X. Yu, C. J. Booth, J. Lian, I. Lazar, S. L. Gamfi, K. Castille, N. Kohya, Y. Nakayama, Y.-C. Liu, et al. (2006)
Mol. Cell. Biol. 26, 5180-5189
   Abstract »    Full Text »    PDF »
Cystic Fibrosis Transmembrane Conductance Regulator Deficiency Exacerbates Islet Cell Dysfunction After {beta}-Cell Injury..
M. S. Stalvey, C. Muller, D. A. Schatz, C. H. Wasserfall, M. L. Campbell-Thompson, D. W. Theriaque, T. R. Flotte, and M. A. Atkinson (2006)
Diabetes 55, 1939-1945
   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
   Abstract »    Full Text »    PDF »
Regulation of the ClC-2 Lung Epithelial Chloride Channel by Glycosylation of SP1.
N. Vij and P. L. Zeitlin (2006)
Am. J. Respir. Cell Mol. Biol. 34, 754-759
   Abstract »    Full Text »    PDF »
Strain-dependent pulmonary gene expression profiles of a cystic fibrosis mouse model..
C. K. Haston, S. Cory, L. Lafontaine, G. Dorion, and M. T. Hallett (2006)
Physiol Genomics 25, 336-345
   Abstract »    Full Text »    PDF »
Distinct pattern of lung gene expression in the Cftr-KO mice developing spontaneous lung disease compared with their littermate controls.
C. Guilbault, J. P. Novak, P. Martin, M.-L. Boghdady, Z. Saeed, M.-C. Guiot, T. J. Hudson, and D. Radzioch (2006)
Physiol Genomics 25, 179-193
   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 »
Assessment of cystic fibrosis transmembrane conductance regulator (CFTR) activity in CFTR-null mice after bone marrow transplantation.
E. M. Bruscia, J. E. Price, E.-C. Cheng, S. Weiner, C. Caputo, E. C. Ferreira, M. E. Egan, and D. S. Krause (2006)
PNAS 103, 2965-2970
   Abstract »    Full Text »    PDF »
Culture of murine nasal epithelia: model for cystic fibrosis.
B. R. Grubb, T. D. Rogers, P. C. Diggs, R. C. Boucher, and L. E. Ostrowski (2006)
Am J Physiol Lung Cell Mol Physiol 290, L270-L277
   Abstract »    Full Text »    PDF »
Respiratory Tract Mucin Genes and Mucin Glycoproteins in Health and Disease.
M. C. Rose and J. A. Voynow (2006)
Physiol Rev 86, 245-278
   Abstract »    Full Text »    PDF »
Blue Native/SDS-PAGE Analysis Reveals Reduced Expression of the mClCA3 Protein in Cystic Fibrosis Knock-out Mice.
F. Brouillard, N. Bensalem, A. Hinzpeter, D. Tondelier, S. Trudel, A. D. Gruber, M. Ollero, and A. Edelman (2005)
Mol. Cell. Proteomics 4, 1762-1775
   Abstract »    Full Text »    PDF »
Disruption of CFTR chloride channel alters mechanical properties and cAMP-dependent Cl- transport of mouse aortic smooth muscle cells.
R. Robert, C. Norez, and F. Becq (2005)
J. Physiol. 568, 483-495
   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 »
CFTR {Delta}F508 mutation has minimal effect on the gene expression profile of differentiated human airway epithelia.
J. Zabner, T. E. Scheetz, H. G. Almabrazi, T. L. Casavant, J. Huang, S. Keshavjee, and P. B. McCray Jr. (2005)
Am J Physiol Lung Cell Mol Physiol 289, L545-L553
   Abstract »    Full Text »    PDF »
Role of Na-K-2Cl cotransporter-1 in gastric secretion of nonacidic fluid and pepsinogen.
N. McDaniel, A. J. Pace, S. Spiegel, R. Engelhardt, B. H. Koller, U. Seidler, and C. Lytle (2005)
Am J Physiol Gastrointest Liver Physiol 289, G550-G560
   Abstract »    Full Text »    PDF »
STa and cGMP stimulate CFTR translocation to the surface of villus enterocytes in rat jejunum and is regulated by protein kinase G.
F. Golin-Bisello, N. Bradbury, and N. Ameen (2005)
Am J Physiol Cell Physiol 289, C708-C716
   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 »
Chloride conductance of CFTR facilitates basal Cl-/HCO3- exchange in the villous epithelium of intact murine duodenum.
J. E. Simpson, L. R. Gawenis, N. M. Walker, K. T. Boyle, and L. L. Clarke (2005)
Am J Physiol Gastrointest Liver Physiol 288, G1241-G1251
   Abstract »    Full Text »    PDF »
Effect of Macrolides on In Vivo Ion Transport across Cystic Fibrosis Nasal Epithelium.
P. M. Barker, D. J. Gillie, M. S. Schechter, and B. K. Rubin (2005)
Am. J. Respir. Crit. Care Med. 171, 868-871
   Abstract »    Full Text »    PDF »
Molecular Physiology and Pathophysiology of Electroneutral Cation-Chloride Cotransporters.
G. Gamba (2005)
Physiol Rev 85, 423-493
   Abstract »    Full Text »    PDF »
Heat-stable enterotoxin of Escherichia coli stimulates a non-CFTR-mediated duodenal bicarbonate secretory pathway.
Z. M. Sellers, D. Childs, J. Y. C. Chow, A. J. Smith, D. L. Hogan, J. I. Isenberg, H. Dong, K. E. Barrett, and V. S. Pratha (2005)
Am J Physiol Gastrointest Liver Physiol 288, G654-G663
   Abstract »    Full Text »    PDF »
Bacterial and Host Factors Implicated in Nasal Carriage of Methicillin-Resistant Staphylococcus aureus in Mice.
B. Gonzalez-Zorn, J. P. M. Senna, L. Fiette, S. Shorte, A. Testard, M. Chignard, P. Courvalin, and C. Grillot-Courvalin (2005)
Infect. Immun. 73, 1847-1851
   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 »
No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis.
A. Werner, M. E. J. Bongers, M. J. Bijvelds, H. R. de Jonge, and H. J. Verkade (2004)
J. Lipid Res. 45, 2277-2286
   Abstract »    Full Text »    PDF »
Electroneutral sodium absorption and electrogenic anion secretion across murine small intestine are regulated in parallel.
L. R. Gawenis, H. Hut, A. G. M. Bot, G. E. Shull, H. R. de Jonge, X. Stien, M. L. Miller, and L. L. Clarke (2004)
Am J Physiol Gastrointest Liver Physiol 287, G1140-G1149
   Abstract »    Full Text »    PDF »
Unravelling salt transport in cystic fibrosis.
P G Noone and K W Southern (2004)
Thorax 59, 916-917
   Full Text »    PDF »
Bacterial Overgrowth in the Cystic Fibrosis Transmembrane Conductance Regulator Null Mouse Small Intestine.
O. Norkina, T. G. Burnett, and R. C. De Lisle (2004)
Infect. Immun. 72, 6040-6049
   Abstract »    Full Text »    PDF »
ALTERED PHARMACOKINETICS OF OMEPRAZOLE IN CYSTIC FIBROSIS KNOCKOUT MICE RELATIVE TO WILD-TYPE MICE.
M. N. Tallman, S. Y. Ali, and P. C. Smith (2004)
Drug Metab. Dispos. 32, 902-905
   Abstract »    Full Text »    PDF »
Targeted Inactivation of Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel Gene Prevents Ischemic Preconditioning in Isolated Mouse Heart.
H. Chen, L. L. Liu, L. L. Ye, C. McGuckin, S. Tamowski, P. Scowen, H. Tian, K. Murray, W. J. Hatton, and D. Duan (2004)
Circulation 110, 700-704
   Abstract »    Full Text »    PDF »
Acidic duodenal pH alters gene expression in the cystic fibrosis mouse pancreas.
S. Kaur, O. Norkina, D. Ziemer, L. C. Samuelson, and R. C. De Lisle (2004)
Am J Physiol Gastrointest Liver Physiol 287, G480-G490
   Abstract »    Full Text »    PDF »
Lateral intercellular space volume as a determinant of CFTR-mediated anion secretion across small intestinal mucosa.
L. R. Gawenis, K. T. Boyle, B. A. Palmer, N. M. Walker, and L. L. Clarke (2004)
Am J Physiol Gastrointest Liver Physiol 286, G1015-G1023
   Abstract »    Full Text »    PDF »
Abnormal Paneth cell granule dissolution and compromised resistance to bacterial colonization in the intestine of CF mice.
L. L. Clarke, L. R. Gawenis, E. M. Bradford, L. M. Judd, K. T. Boyle, J. E. Simpson, G. E. Shull, H. Tanabe, A. J. Ouellette, C. L. Franklin, et al. (2004)
Am J Physiol Gastrointest Liver Physiol 286, G1050-G1058
   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 »
Curcumin, a Major Constituent of Turmeric, Corrects Cystic Fibrosis Defects.
M. E. Egan, M. Pearson, S. A. Weiner, V. Rajendran, D. Rubin, J. Glockner-Pagel, S. Canny, K. Du, G. L. Lukacs, and M. J. Caplan (2004)
Science 304, 600-602
   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 »
Role for Cystic Fibrosis Transmembrane Conductance Regulator Protein in a Glutathione Response to Bronchopulmonary Pseudomonas Infection.
B. J. Day, A. M. van Heeckeren, E. Min, and L. W. Velsor (2004)
Infect. Immun. 72, 2045-2051
   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 »
Mucociliary transport determined by in vivo microdialysis in the airways of normal and CF mice.
B. R. Grubb, J. H. Jones, and R. C. Boucher (2004)
Am J Physiol Lung Cell Mol Physiol 286, L588-L595
   Abstract »    Full Text »    PDF »
Protection of Cftr knockout mice from acute lung infection by a helper-dependent adenoviral vector expressing Cftr in airway epithelia.
D. R. Koehler, U. Sajjan, Y.-H. Chow, B. Martin, G. Kent, A. K. Tanswell, C. McKerlie, J. F. Forstner, and J. Hu (2003)
PNAS 100, 15364-15369
   Abstract »    Full Text »    PDF »
Surface-lining layer of airways in cystic fibrosis mice.
M. Geiser and S. Bastian (2003)
Am J Physiol Lung Cell Mol Physiol 285, L1277-L1285
   Abstract »    Full Text »    PDF »
Acute regulation of the SLC26A3 congenital chloride diarrhoea anion exchanger (DRA) expressed in Xenopus oocytes.
M. N Chernova, L. Jiang, B. E Shmukler, C. W Schweinfest, P. Blanco, S. D Freedman, A. K Stewart, and S. L Alper (2003)
J. Physiol. 549, 3-19
   Abstract »    Full Text »    PDF »
GENETICALLY ALTERED MOUSE MODELS: THE GOOD, THE BAD, AND THE UGLY.
T. Thyagarajan, S. Totey, M. J. S. Danton, and A. B. Kulkarni (2003)
Critical Reviews in Oral Biology & Medicine 14, 154-174
   Abstract »    Full Text »    PDF »
Altered pH Regulation During Enamel Development in the Cystic Fibrosis Mouse Incisor.
W. Sui, C. Boyd, and J.T. Wright (2003)
Journal of Dental Research 82, 388-392
   Abstract »    Full Text »    PDF »
Novel MUC1 splice variants contribute to mucin overexpression in CFTR-deficient mice.
A. M. Hinojosa-Kurtzberg, M. E. V. Johansson, C. S. Madsen, G. C. Hansson, and S. J. Gendler (2003)
Am J Physiol Gastrointest Liver Physiol 284, G853-G862
   Abstract »    Full Text »    PDF »
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
   Abstract »    Full Text »    PDF »
CFTR-dependent and -independent swelling-activated K+ currents in primary cultures of mouse nephron.
R. Belfodil, H. Barriere, I. Rubera, M. Tauc, C. Poujeol, M. Bidet, and P. Poujeol (2003)
Am J Physiol Renal Physiol 284, F812-F828
   Abstract »    Full Text »    PDF »
Transcriptional Adaptation to Cystic Fibrosis Transmembrane Conductance Regulator Deficiency.
Y. Xu, J. C. Clark, B. J. Aronow, C. R. Dey, C. Liu, J. L. Wooldridge, and J. A. Whitsett (2003)
J. Biol. Chem. 278, 7674-7682
   Abstract »    Full Text »    PDF »
A novel chloride conductance activated by extracellular ATP in mouse parotid acinar cells.
J. Arreola and J. E Melvin (2003)
J. Physiol. 547, 197-208
   Abstract »    Full Text »    PDF »
Loci of intestinal distress in cystic fibrosis knockout mice.
C. K. Haston and L.-C. Tsui (2003)
Physiol Genomics 12, 79-84
   Abstract »    Full Text »    PDF »
Pleural surface fluorescence measurement of Na+ and Cl- transport across the air space-capillary barrier.
J. Jiang, Y. Song, C. Bai, B. H. Koller, M. A. Matthay, and A. S. Verkman (2003)
J Appl Physiol 94, 343-352
   Abstract »    Full Text »    PDF »
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
   Abstract »    Full Text »    PDF »
Targeted Inactivation of the Mouse Guanylin Gene Results in Altered Dynamics of Colonic Epithelial Proliferation.
K. A. Steinbrecher, S. A. Wowk, J. A. Rudolph, D. P. Witte, and M. B. Cohen (2002)
Am. J. Pathol. 161, 2169-2178
   Abstract »    Full Text »    PDF »
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
   Abstract »    Full Text »    PDF »
Cystic Fibrosis Transmembrane Regulator Gene (CFTR) is Associated with Abnormal Enamel Formation.
C.K. Arquitt, C. Boyd, and J.T. Wright (2002)
Journal of Dental Research 81, 492-496
   Abstract »    Full Text »    PDF »
The severe G480C cystic fibrosis mutation, when replicated in the mouse, demonstrates mistrafficking, normal survival and organ-specific bioelectrics.
P. Dickinson, S. N. Smith, S. Webb, F. M. Kilanowski, I. J. Campbell, M. S. Taylor, D. J. Porteous, R. Willemsen, H. R. de Jonge, R. Farley, et al. (2002)
Hum. Mol. Genet. 11, 243-251
   Abstract »    Full Text »    PDF »
Electrolyte Transport in the Mammalian Colon: Mechanisms and Implications for Disease.
K. Kunzelmann and M. Mall (2002)
Physiol Rev 82, 245-289
   Abstract »    Full Text »    PDF »
A2 adenosine receptors regulate CFTR through PKA and PLA2.
B. R. Cobb, F. Ruiz, C. M. King, J. Fortenberry, H. Greer, T. Kovacs, E. J. Sorscher, and J. P. Clancy (2002)
Am J Physiol Lung Cell Mol Physiol 282, L12-L25
   Abstract »    Full Text »    PDF »
Animal models of chronic obstructive pulmonary disease.
P A Dawkins and R A Stockley (2001)
Thorax 56, 972-977
   Full Text »    PDF »
Partial correction of defective Cl- secretion in cystic fibrosis epithelial cells by an analog of squalamine.
C. Jiang, E. R. Lee, M. B. Lane, Y.-F. Xiao, D. J. Harris, and S. H. Cheng (2001)
Am J Physiol Lung Cell Mol Physiol 281, L1164-L1172
   Abstract »    Full Text »    PDF »
Changes in the exocrine pancreas secondary to altered small intestinal function in the CF mouse.
R. C. De Lisle, K. S. Isom, D. Ziemer, and C. U. Cotton (2001)
Am J Physiol Gastrointest Liver Physiol 281, G899-G906
   Abstract »    Full Text »    PDF »
CFTR disruption impairs cAMP-dependent Cl- secretion in primary cultures of mouse cortical collecting ducts.
M. Bens, J.-P. D. Van Huyen, F. Cluzeaud, J. Teulon, and A. Vandewalle (2001)
Am J Physiol Renal Physiol 281, F434-F442
   Abstract »    Full Text »    PDF »
Enhanced Susceptibility to Pulmonary Infection with Burkholderia cepacia in Cftr{-}/{-} Mice.
U. Sajjan, G. Thanassoulis, V. Cherapanov, A. Lu, C. Sjolin, B. Steer, Y. J. Wu, O. D. Rotstein, G. Kent, C. McKerlie, et al. (2001)
Infect. Immun. 69, 5138-5150
   Abstract »    Full Text »    PDF »
Antioxidant imbalance in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice.
L. W. Velsor, A. van Heeckeren, and B. J. Day (2001)
Am J Physiol Lung Cell Mol Physiol 281, L31-L38
   Abstract »    Full Text »    PDF »
Partial restoration of defective chloride conductance in {Delta}F508 CF mice by trimethylamine oxide.
H. Fischer, N. Fukuda, P. Barbry, B. Illek, C. Sartori, and M. A. Matthay (2001)
Am J Physiol Lung Cell Mol Physiol 281, L52-L57
   Abstract »    Full Text »    PDF »
Characteristics of rodent intestinal mucin Muc3 and alterations in a mouse model of human cystic fibrosis.
I. A. Khatri, C. Ho, R. D. Specian, and J. F. Forstner (2001)
Am J Physiol Gastrointest Liver Physiol 280, G1321-G1330
   Abstract »    Full Text »    PDF »
Cloning and Functional Characterization of Two Murine Uridine Nucleotide Receptors Reveal a Potential Target for Correcting Ion Transport Deficiency in Cystic Fibrosis Gallbladder.
E. R. Lazarowski, L. G. Rochelle, W. K. O'Neal, C. M. P. Ribeiro, B. R. Grubb, V. Zhang, T. K. Harden, and R. C. Boucher (2001)
J. Pharmacol. Exp. Ther. 297, 43-49
   Abstract »    Full Text »
A src-like kinase activates outwardly rectifying chloride channels in CFTR-defective lymphocytes.
A. LEPPLE-WIENHUES, U. WIELAND, T. LAUN, L. HEIL, M. STERN, and F. LANG (2001)
FASEB J 15, 927-931
   Abstract »    Full Text »    PDF »


To Advertise     Find Products

Science. ISSN 0036-8075 (print), 1095-9203 (online)