Cftr

Summary

Gene Symbol: Cftr
Description: cystic fibrosis transmembrane conductance regulator
Alias: AW495489, Abcc7, cystic fibrosis transmembrane conductance regulator, ATP-binding cassette sub-family C member 7, ATP-binding cassette transporter sub-family C member 7, ATP-binding cassette, subfamily c, member 7, cAMP-dependent chloride channel, channel conductance-controlling ATPase, cystic fibrosis transmembrane conductance regulator homolog, cystic fibrosis transmembrane conductance regulator homolog; ATP-binding cassette, subfamily c, member 7
Species: mouse

Top Publications

  1. pmc A mouse model for the delta F508 allele of cystic fibrosis
    B G Zeiher
    Howard Hughes Medical Institute, Department of Internal Medicine, University of Iowa College of Medicine, Iowa City 52242, USA
    J Clin Invest 96:2051-64. 1995
  2. ncbi Loci of intestinal distress in cystic fibrosis knockout mice
    Christina K Haston
    Program of Genetics and Genomic Biology, Research Institute, The Hospital for Sick Children, and Department of Molecular and Medical Genetics, University of Toronto, Ontario, Canada
    Physiol Genomics 12:79-84. 2003
  3. ncbi Blue native/SDS-PAGE analysis reveals reduced expression of the mClCA3 protein in cystic fibrosis knock-out mice
    Franck Brouillard
    INSERM U467, 156 rue de Vaugirard, Paris F 75015 and Université Paris Descartes, Faculte de Medecine, 15 rue Ecole de Médecine, Paris F 75005, France
    Mol Cell Proteomics 4:1762-75. 2005
  4. pmc Spontaneous rescue from cystic fibrosis in a mouse model
    Nikoletta Charizopoulou
    Klinische Forschergruppe, OE 6710, Medizinische Hochschule Hannover, D 30625 Hannover, Germany
    BMC Genet 7:18. 2006
  5. ncbi Effects of laxative and N-acetylcysteine on mucus accumulation, bacterial load, transit, and inflammation in the cystic fibrosis mouse small intestine
    Robert C De Lisle
    Anatomy and Cell Biology, University of Kansas School of Medicine, Kansas City, KS 66160, USA
    Am J Physiol Gastrointest Liver Physiol 293:G577-84. 2007
  6. pmc Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with betaENaC-overexpressing mice
    Marcus A Mall
    Division of Pediatric Pulmonology and Cystic Fibrosis Center, Department of Pediatrics III, University of Heidelberg, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany
    J Biol Chem 285:26945-55. 2010
  7. ncbi A severe phenotype in mice with a duplication of exon 3 in the cystic fibrosis locus
    W K O'Neal
    Institute for Molecular Genetics, Baylor College of Medicine, Houston, TX 77030
    Hum Mol Genet 2:1561-9. 1993
  8. ncbi Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR
    L Zhou
    Children s Hospital Medical Center, Division of Pulmonary Biology, Cincinnati, OH 45229 3039
    Science 266:1705-8. 1994
  9. pmc A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo
    P J French
    Department of Cell Biology, Erasmus University, Rotterdam, The Netherlands
    J Clin Invest 98:1304-12. 1996
  10. pmc Generation of a conditional null allele for Cftr in mice
    Craig A Hodges
    Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106, USA
    Genesis 46:546-52. 2008

Research Grants

  1. Cholesterol transport regulation in cystic fibrosis
    Thomas Kelley; Fiscal Year: 2007
  2. TRANSGENIC MOUSE MODELS OF PANCREATIC EXOCRINE FUNCTION
    ROBERT DE LISLE; Fiscal Year: 2003
  3. PATHOGENEISIS OF CYSTIC FIBROSIS IN THE GI SYSTEM
    ROBERT DE LISLE; Fiscal Year: 2003
  4. ALTERED EPITHELIAL STAT1 DEPENDENT SIGNALING IN CYSTIC F
    Thomas Kelley; Fiscal Year: 2003
  5. Redox control of Nrf2/ARE by thioredoxin and GSH
    Jason Hansen; Fiscal Year: 2005
  6. IDENTIFICATION OF BIOCHEMICAL ABNORMALITIES IN PCD CILIA
    LAWRENCE OSTROWSKI; Fiscal Year: 2002
  7. CHOLESTEROL TRANSPORT IN GALLBLADDER EPITHELIAL CELLS
    Rahul Kuver; Fiscal Year: 2003
  8. MUCIN SECRETION IN COLUMNAR EPITHELIAL CELLS
    Rahul Kuver; Fiscal Year: 2003
  9. A Ciliated Cell-Specific Promoter for Gene Therapy of CF
    LAWRENCE OSTROWSKI; Fiscal Year: 2005
  10. P. aeruginosa lung disease in CF
    Anna Van Heeckeren; Fiscal Year: 2006

Detail Information

Publications156 found, 100 shown here

  1. pmc A mouse model for the delta F508 allele of cystic fibrosis
    B G Zeiher
    Howard Hughes Medical Institute, Department of Internal Medicine, University of Iowa College of Medicine, Iowa City 52242, USA
    J Clin Invest 96:2051-64. 1995
    The most common cause of cystic fibrosis is a mutation that deletes phenylalanine 508 in cystic fibrosis transmembrane conductance regulator (CFTR)...
  2. ncbi Loci of intestinal distress in cystic fibrosis knockout mice
    Christina K Haston
    Program of Genetics and Genomic Biology, Research Institute, The Hospital for Sick Children, and Department of Molecular and Medical Genetics, University of Toronto, Ontario, Canada
    Physiol Genomics 12:79-84. 2003
    ..The survival of female mice, both F2 CF and F2 control, was linked to loci on chromosomes 3 and 5. The identification of additional putative CF modifier loci may permit further genetic dissection of the complex CF phenotype...
  3. ncbi Blue native/SDS-PAGE analysis reveals reduced expression of the mClCA3 protein in cystic fibrosis knock-out mice
    Franck Brouillard
    INSERM U467, 156 rue de Vaugirard, Paris F 75015 and Université Paris Descartes, Faculte de Medecine, 15 rue Ecole de Médecine, Paris F 75005, France
    Mol Cell Proteomics 4:1762-75. 2005
    ..disorder caused by mutation of a gene encoding a multifunctional transmembrane protein, the cystic fibrosis transmembrane conductance regulator (CFTR), located in the apical membrane of epithelial cells lining exocrine glands...
  4. pmc Spontaneous rescue from cystic fibrosis in a mouse model
    Nikoletta Charizopoulou
    Klinische Forschergruppe, OE 6710, Medizinische Hochschule Hannover, D 30625 Hannover, Germany
    BMC Genet 7:18. 2006
    ..In order to further investigate whether these genetic differences have an impact on the disease phenotype of cystic fibrosis we characterised the phenotype of the two inbred strains...
  5. ncbi Effects of laxative and N-acetylcysteine on mucus accumulation, bacterial load, transit, and inflammation in the cystic fibrosis mouse small intestine
    Robert C De Lisle
    Anatomy and Cell Biology, University of Kansas School of Medicine, Kansas City, KS 66160, USA
    Am J Physiol Gastrointest Liver Physiol 293:G577-84. 2007
    ..These results suggest that both mucus accumulation and slowed interdigestive small intestinal transit contribute to SIBO in the CF intestine...
  6. pmc Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with betaENaC-overexpressing mice
    Marcus A Mall
    Division of Pediatric Pulmonology and Cystic Fibrosis Center, Department of Pediatrics III, University of Heidelberg, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany
    J Biol Chem 285:26945-55. 2010
    ..To investigate this apparent paradox, we compared the airway phenotype (nasal versus tracheal) of Liddle with CFTR-null, betaENaC-Tg, and double mutant mice...
  7. ncbi A severe phenotype in mice with a duplication of exon 3 in the cystic fibrosis locus
    W K O'Neal
    Institute for Molecular Genetics, Baylor College of Medicine, Houston, TX 77030
    Hum Mol Genet 2:1561-9. 1993
    ..Thus, an additional mouse model for CF has been generated that should prove useful for the understanding of the pathogenesis and the development of treatments for CF...
  8. ncbi Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR
    L Zhou
    Children s Hospital Medical Center, Division of Pulmonary Biology, Cincinnati, OH 45229 3039
    Science 266:1705-8. 1994
    Cystic fibrosis (CF) is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR)...
  9. pmc A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo
    P J French
    Department of Cell Biology, Erasmus University, Rotterdam, The Netherlands
    J Clin Invest 98:1304-12. 1996
    ..fibrosis patients inhibits maturation and transfer to the plasma membrane of the mutant cystic fibrosis transmembrane conductance regulator (CFTR)...
  10. pmc Generation of a conditional null allele for Cftr in mice
    Craig A Hodges
    Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106, USA
    Genesis 46:546-52. 2008
    The cystic fibrosis transmembrane conductance regulator (CFTR) gene encodes a cAMP-regulated chloride channel that is important in controlling the exchange of fluid and electrolytes across epithelial cells...
  11. pmc Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations
    S J Delaney
    Centre for Molecular and Cellular Biology, University of Queensland, Brisbane, Australia
    EMBO J 15:955-63. 1996
    We have generated a mouse carrying the human G551D mutation in the cystic fibrosis transmembrane conductance regulator gene (CFTR) by a one-step gene targeting procedure...
  12. ncbi Production of a severe cystic fibrosis mutation in mice by gene targeting
    R Ratcliff
    Wellcome CRC Institute of Cancer and Developmental Biology, University of Cambridge, UK
    Nat Genet 4:35-41. 1993
    ..in embryonic stem cells to introduce an HPRT mini-gene into the coding sequence of the murine cystic fibrosis gene (cftr)...
  13. pmc Cystic fibrosis mice lacking Muc1 have reduced amounts of intestinal mucus
    R R Parmley
    Mayo Clinic Scottsdale, Department of Biochemistry and Molecular Biology, Scottsdale, Arizona 85259, USA
    J Clin Invest 102:1798-806. 1998
    ..mucin RNA and protein expression in the gastrointestinal tract of inbred CF transmembrane conductance regulator (CFTR) knockout (CF) mice and correlated expression with histological analyses of tissues...
  14. pmc Macrophages directly contribute to the exaggerated inflammatory response in cystic fibrosis transmembrane conductance regulator-/- mice
    Emanuela M Bruscia
    Yale University School of Medicine, Department of Pediatrics, 333 Cedar Street, PO Box 208064, New Haven, CT 06520 8035, USA
    Am J Respir Cell Mol Biol 40:295-304. 2009
    ....
  15. pmc Reduced NHE3-mediated Na+ absorption increases survival and decreases the incidence of intestinal obstructions in cystic fibrosis mice
    Emily M Bradford
    Department of Molecular Genetics, Biochemistry and Microbiology, University of Cincinnati, College of Medicine, 231 Albert Sabin Way, ML0524, Cincinnati, OH 45267 0524, USA
    Am J Physiol Gastrointest Liver Physiol 296:G886-98. 2009
    In cystic fibrosis, impaired secretion resulting from loss of activity of the cystic fibrosis transmembrane conductance regulator (CFTR) causes dehydration of intestinal contents and life-threatening obstructions...
  16. pmc Ion transport across CF and normal murine olfactory and ciliated epithelium
    B R Grubb
    Cystic Fibrosis Pulmonary Research and Treatment Center, 7011 Thurston Bowles Bldg, CB 7248, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolia 27599 7248, USA
    Am J Physiol Cell Physiol 296:C1301-9. 2009
    ..Thus, while the murine CF nasal epithelium is a valuable model for CF studies, the bioelectrics are likely dominated by the signals from the OE, and assays of the nasopharynx may be more specific for studying the ciliated epithelium...
  17. pmc NMR evidence for differential phosphorylation-dependent interactions in WT and DeltaF508 CFTR
    Voula Kanelis
    Program in Molecular Structure and Function, Hospital for Sick Children, Toronto, Ontario, Canada
    EMBO J 29:263-77. 2010
    The most common cystic fibrosis (CF)-causing mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) is deletion of Phe508 (DeltaF508) in the first of two nucleotide-binding domains (NBDs)...
  18. pmc Transgenic hCFTR expression fails to correct β-ENaC mouse lung disease
    B R Grubb
    Cystic Fibrosis Pulmonary Research and Treatment Center, The University of North Carolina at Chapel Hill, Chapel Hill, NC 27599 7248, USA
    Am J Physiol Lung Cell Mol Physiol 302:L238-47. 2012
    ..1) To determine if transgenic overexpression of wild-type (WT) human CFTR (hCFTR) accelerated Cl(-) secretion and regulated Na(+) absorption in murine airways, we utilized a Clara cell ..
  19. ncbi G551D cystic fibrosis mice exhibit abnormal regulation of inflammation in lungs and macrophages
    G R Thomas
    Centre for Molecular Biology, Department of Biochemistry and Microbiology, University of Queensland, St Lucia, Australia
    J Immunol 164:3870-7. 2000
    ..These results provide evidence that the pathology of CF relates to abnormal regulation of the immune system...
  20. pmc Cftr and ENaC ion channels mediate NaCl absorption in the mouse submandibular gland
    Marcelo A Catalán
    Department of Pharmacology and Physiology, University of Rochester Medical Center, Rochester, NY 14620, USA
    J Physiol 588:713-24. 2010
    Cystic fibrosis is caused by mutations in CFTR, the cystic fibrosis transmembrane conductance regulator gene...
  21. pmc Revisiting the role of cystic fibrosis transmembrane conductance regulator and counterion permeability in the pH regulation of endocytic organelles
    Herve Barriere
    Department of Physiology, McGill University, Montreal, Quebec, H3G 1Y6, Canada
    Mol Biol Cell 20:3125-41. 2009
    ..defective organellar pH regulation, caused by impaired counterion conductance of the mutant cystic fibrosis transmembrane conductance regulator (CFTR), remains highly controversial in epithelia and macrophages...
  22. ncbi Dietary changes improve survival of CFTR S489X homozygous mutant mouse
    E A Eckman
    Department of Pediatrics, Case Western Reserve University, Cleveland, Ohio 44106, USA
    Am J Physiol 269:L625-30. 1995
    Over 90% of untreated CFTR S489X homozygous (CF) mutant mice reportedly die of intestinal obstruction by 40 days of age, significantly limiting their usefulness as a model for the human disease...
  23. ncbi The severe G480C cystic fibrosis mutation, when replicated in the mouse, demonstrates mistrafficking, normal survival and organ-specific bioelectrics
    Paul Dickinson
    MRC Human Genetics Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, UK
    Hum Mol Genet 11:243-51. 2002
    The majority of cystic fibrosis patients produce a mutant form of CFTR (DeltaF508) which has been shown to be mislocalized in both humans and mice...
  24. ncbi Detection of modifier loci influencing the lung phenotype of cystic fibrosis knockout mice
    Christina K Haston
    Program of Genetics and Genomic Biology, The Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada
    Mamm Genome 13:605-13. 2002
    ..disease associated with cystic fibrosis (CF) cannot be explained by the genotype of the cystic fibrosis transmembrane conductance regulator (CFTR) locus alone...
  25. pmc Assessment of cystic fibrosis transmembrane conductance regulator (CFTR) activity in CFTR-null mice after bone marrow transplantation
    Emanuela M Bruscia
    Department of Laboratory Medicine, Yale University School of Medicine, 330 Cedar Street, New Haven, CT 06520, USA
    Proc Natl Acad Sci U S A 103:2965-70. 2006
    ..We investigate whether, after transplantation of cystic fibrosis transmembrane conductance regulator (CFTR)-positive BM-derived cells, BM-derived GI and airway epithelial cells can provide ..
  26. ncbi Amelioration of cystic fibrosis intestinal mucous disease in mice by restoration of mCLCA3
    Fiona D Young
    University of Western Ontario, London, Ontario, Canada Lawson Health Research Institute, London, Ontario, Canada
    Gastroenterology 133:1928-37. 2007
    Mice deficient of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) exhibit severe intestinal lesions, particularly mucous overproduction/secretion and accumulation, which is similar to meconium ileus in CF patients...
  27. ncbi An animal model for cystic fibrosis made by gene targeting
    J N Snouwaert
    Department of Medicine, University of North Carolina, Chapel Hill 27599 7020
    Science 257:1083-8. 1992
    ..gene encoding a cyclic adenosine monophosphate-dependent chloride ion channel known as the cystic fibrosis transmembrane conductance regulator (CFTR)...
  28. ncbi Cystic fibrosis in the mouse by targeted insertional mutagenesis
    J R Dorin
    MRC Human Genetics Unit, Western General Hospital, Edinburgh, UK
    Nature 359:211-5. 1992
    ..The mouse cystic fibrosis transmembrane conductance regulator gene was disrupted in embryonal stem cells using an insertional gene targeting vector...
  29. ncbi Bioelectric characteristics of exon 10 insertional cystic fibrosis mouse: comparison with humans
    S N Smith
    Ion Transport Unit, National Heart and Lung Institute, London, United Kingdom
    Am J Physiol 268:C297-307. 1995
    ..We conclude that the majority of the salient electrophysiological features of CF required for studies of pathogenesis or testing of new treatments are present in these cf/cf mice...
  30. ncbi Molecular pathophysiology of cystic fibrosis based on the rescued knockout mouse model
    J C Cohen
    Department of Medicine, Louisiana State University, School of Medicine, New Orleans, Louisiana 70112, USA
    Mol Genet Metab 64:108-18. 1998
    b>Cystic fibrosis transmembrane conductance regulator (cftr) gene mutations are thought to result in cystic fibrosis due to an absence of the protein's chloride channel...
  31. ncbi Antioxidant imbalance in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice
    L W Velsor
    Department of Medicine, National Jewish Medical and Research Center, Denver, Colorado 80206, USA
    Am J Physiol Lung Cell Mol Physiol 281:L31-8. 2001
    Recent studies suggest that the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) protein modulates epithelial reduced glutathione (GSH) transport and when defective creates an antioxidant imbalance...
  32. ncbi Host defense against Pseudomonas aeruginosa requires ceramide-rich membrane rafts
    H Grassme
    Department of Molecular Biology, University of Essen, Essen, Germany
    Nat Med 9:322-30. 2003
    ..Our findings show that ceramide-enriched membrane platforms are central to the host defense against this potentially lethal pathogen...
  33. ncbi Role of Cftr genotype in the response to chronic Pseudomonas aeruginosa lung infection in mice
    Anna M van Heeckeren
    Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106 4948, USA
    Am J Physiol Lung Cell Mol Physiol 287:L944-52. 2004
    Patients with cystic fibrosis have a lesion in the cystic fibrosis transmembrane conductance regulator gene (CFTR), which is associated with abnormal regulation of other ion channels, abnormal glycosylation of secreted and cell surface ..
  34. pmc Bacterial overgrowth in the cystic fibrosis transmembrane conductance regulator null mouse small intestine
    Oxana Norkina
    Anatomy and Cell Biology, University of Kansas School of Medicine, Kansas City, Kansas 66160, USA
    Infect Immun 72:6040-9. 2004
    ..These data suggest that bacterial overgrowth in the CF mouse small intestine has a role in inflammation and contributes to the failure to thrive in this mouse model of CF...
  35. ncbi Role of IL-10 deficiency in excessive nuclear factor-kappaB activation and lung inflammation in cystic fibrosis transmembrane conductance regulator knockout mice
    Aicha Saadane
    Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA
    J Allergy Clin Immunol 115:405-11. 2005
    ..Patients with cystic fibrosis (CF) and CF transmembrane conductance regulator knockout (CF-KO) mice are deficient in pulmonary IL-10 and have excessive inflammatory response to Pseudomonas aeruginosa infection...
  36. ncbi Functional genomic responses to cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR(delta508) in the lung
    Yan Xu
    Division of Pulmonary Biology, Department of Pediatrics, Cincinnati Children s Hospital Medical Center and the University of Cincinnati College of Medicine, Cincinnati, Ohio 45229 3039, USA
    J Biol Chem 281:11279-91. 2006
    ..CF), a common lethal pulmonary disorder in Caucasians, is caused by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR) that disturbs fluid homeostasis and host defense in target organs...
  37. ncbi Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis
    Nicole M White
    Department of Pediatrics and Pharmacology, Case Western Reserve University and Rainbow Babies and Children s Hospital, Cleveland, Ohio 44106, USA
    Am J Physiol Lung Cell Mol Physiol 292:L476-86. 2007
    ..increase in plasma membrane cholesterol content in both cultured CF cells and in nasal tissue excised from cftr(-/-) mice...
  38. doi Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis
    Volker Teichgräber
    Department of Molecular Biology, Hufelandstrasse 55, University of Duisburg Essen, 45122 Essen, Germany
    Nat Med 14:382-91. 2008
    ..disorder cystic fibrosis, yet the molecular mechanisms leading from the mutation of cystic fibrosis transmembrane conductance regulator (CFTR) to lung infection are still unclear...
  39. pmc Congenital tracheal malformation in cystic fibrosis transmembrane conductance regulator-deficient mice
    Elise Bonvin
    UPMC Universite Paris 06, F 75005 Paris, France
    J Physiol 586:3231-43. 2008
    ..that alterations in the extrathoracic airways, particularly in the trachea that expresses high levels of CFTR (CF transmembrane conductance regulator), may contribute to respiratory dysfunction...
  40. ncbi Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis
    L L Clarke
    Department of Medicine, University of North Carolina, Chapel Hill 27514
    Science 257:1125-8. 1992
    The cystic fibrosis transmembrane conductance regulator (CFTR) gene encodes an adenosine 3',5'-monophosphate (cyclic AMP)-activated chloride channel...
  41. ncbi Cloning the mouse homolog of the human cystic fibrosis transmembrane conductance regulator gene
    F Tata
    Department of Biochemistry and Molecular Genetics, St Mary s Hospital Medical School, Imperial College, London, United Kingdom
    Genomics 10:301-7. 1991
    The cystic fibrosis transmembrane conductance regulator is encoded by the gene known to be mutated in patients with cystic fibrosis...
  42. pmc Toward an animal model of cystic fibrosis: targeted interruption of exon 10 of the cystic fibrosis transmembrane regulator gene in embryonic stem cells
    B H Koller
    Department of Pathology, University of North Carolina, Chapel Hill 27599 7525
    Proc Natl Acad Sci U S A 88:10730-4. 1991
    ..8 kilobases of DNA spanning exon 10 of the mouse cystic fibrosis transmembrane regulator (CFTR) gene in which part of the exon has been replaced by two neomycin-resistance (Neo) genes driven by different ..
  43. ncbi Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA
    J R Riordan
    Department of Biochemistry, Hospital for Sick Children, Toronto, Ontario, Canada
    Science 245:1066-73. 1989
    ..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...
  44. ncbi Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model
    S E Gabriel
    Department of Medicine, University of North Carolina, Chapel Hill 27599
    Science 266:107-9. 1994
    ..CF mice that expressed no CF transmembrane conductance regulator (CFTR) protein did not secrete fluid in response to CT...
  45. ncbi Lung disease in the cystic fibrosis mouse exposed to bacterial pathogens
    D J Davidson
    MRC Human Genetics Unit, Western General Hospital, Edinburgh, UK
    Nat Genet 9:351-7. 1995
    ....
  46. ncbi Generation and characterization of a delta F508 cystic fibrosis mouse model
    W H Colledge
    Wellcome CRC Institute of Cancer and Developmental Biology, University of Cambridge, UK
    Nat Genet 10:445-52. 1995
    ..generated mice carrying the most common mutation in cystic fibrosis (CF), delta F508, within the cystic fibrosis (Cftr) gene. Mutant animals show pathological and electrophysiological changes consistent with a CF phenotype...
  47. pmc A mouse model for the cystic fibrosis delta F508 mutation
    J H van Doorninck
    MGC Department of Cell Biology and Genetics, Erasmus University, Rotterdam, The Netherlands
    EMBO J 14:4403-11. 1995
    Most cystic fibrosis (CF) patients produce a mutant form (delta F508) of the cystic fibrosis transmembrane conductance regulator (CFTR), which is not properly processed in normal cells but is active as a chloride channel in several ..
  48. ncbi Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor
    R Rozmahel
    Department of Molecular Genetics, The University of Toronto, Ontario, Canada
    Nat Genet 12:280-7. 1996
    Mice that have been made deficient for the cystic fibrosis transmembrane conductance regulator (Cftr) usually die of intestinal obstruction...
  49. ncbi Phenotypic abnormalities in long-term surviving cystic fibrosis mice
    G Kent
    Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada
    Pediatr Res 40:233-41. 1996
    Mouse models for cystic fibrosis (CF) with no CFTR function (Cftr-/-) have the disadvantage that most animals die of intestinal obstruction shortly after weaning...
  50. ncbi Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice
    J M Zahm
    Institut National de la Santé et de la Recherche Médicale U 314, 1FR53 CHU Maison Blanche, Reims, France
    Am J Physiol 272:C853-9. 1997
    In cystic fibrosis (CF), whether cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction leads to decreased mucociliary clearance and mucus hypersecretion, before bacterial infection, remains an open question...
  51. ncbi Immuno and functional characterization of CFTR in submandibular and pancreatic acinar and duct cells
    W Zeng
    Department of Physiology, University of Texas Southwestern Medical Center, Dallas 75235, USA
    Am J Physiol 273:C442-55. 1997
    Cystic fibrosis results from defective Cl- channel activity mediated by the cystic fibrosis transmembrane conductance regulator (CFTR) gene product...
  52. ncbi Intestinal physiology and pathology in gene-targeted mouse models of cystic fibrosis
    B R Grubb
    Cystic Fibrosis Pulmonary Research and Clinical Treatment Center, University of North Carolina, Chapel Hill 27599, USA
    Am J Physiol 273:G258-66. 1997
    ..Cloning of the gene causing CF was accomplished in 1989, and the protein product, cystic fibrosis transmembrane conductance regulator (CFTR), has been conclusively shown to be an adenosine 3',5'-cyclic monophosphate (cAMP)-..
  53. pmc Lung disease in mice with cystic fibrosis
    G Kent
    Research Institute, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada
    J Clin Invest 100:3060-9. 1997
    ..The utility of the CFTR-knockout mouse in studying the pathogenesis of cystic fibrosis has been limited because of their failure, despite ..
  54. ncbi Tissue-specific in vivo transcription start sites of the human and murine cystic fibrosis genes
    N L White
    Nuffield Department of Clinical Biochemistry and Imperial Cancer Research Fund Laboratories, Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, Oxford OX3 9DS, UK
    Hum Mol Genet 7:363-9. 1998
    The in vivo transcription start sites of the human cystic fibrosis transmembrane conductance regulator gene ( CFTR ) and its murine homologue ( Cftr ) have been mapped in a range of tissues using the technique of 5' rapid amplification ..
  55. ncbi Impaired ability of Cftr knockout mice to control lung infection with Pseudomonas aeruginosa
    D Gosselin
    Centre for the Study of Host Resistance, McGill University, Montreal, Quebec, Canada
    Am J Respir Crit Care Med 157:1253-62. 1998
    The present study was aimed at investigating the innate susceptibility of C57BL/6-Cftrunc/Cftrunc knockout [B6-Cftr (-/-)] mice to pulmonary infection with Pseudomonas aeruginosa. Our results indicate that 58...
  56. ncbi In-frame elimination of exon 10 in Cftrtm1Unc CF mice
    Z Xu
    Cardiovascular Research Institute, University of California, San Francisco, CA 94143, USA
    Gene 211:117-23. 1998
    ..In the present study, CFTR mRNA expression was examined in different tissues from one CF mouse model that contains a disruption in exon 10 ..
  57. ncbi Pathophysiology of gene-targeted mouse models for cystic fibrosis
    B R Grubb
    Cystic Fibrosis Pulmonary Research and Treatment Center, School of Medicine, The University of North Carolina at Chapel Hill, USA
    Physiol Rev 79:S193-214. 1999
    ..Reasons for these unexpected findings are discussed. This paper also reviews pharmacotherapeutic and gene therapeutic studies in the various mouse models...
  58. ncbi Functional analysis of cis-acting elements regulating the alternative splicing of human CFTR exon 9
    M Niksic
    International Centre for Genetic Engineering and Biotechnology ICGEB, Padriciano 99, 34012, Trieste, Italy
    Hum Mol Genet 8:2339-49. 1999
    The rate of exon 9 exclusion from the cystic fibrosis transmembrane conductance regulator (CFTR) mRNA is associated with monosymptomatic forms of cystic fibrosis...
  59. ncbi CFTR modulates lung secretory cell proliferation and differentiation
    J E Larson
    Laboratory of Molecular Genetics, Alton Ochsner Medical Foundation, New Orleans 70121, USA
    Am J Physiol Lung Cell Mol Physiol 279:L333-41. 2000
    We have permanently reversed the lethal phenotype in the cystic fibrosis (CF) transmembrane conductance regulator (CFTR)-deficient (knockout) mouse after in utero gene therapy with an adenovirus containing the cftr gene...
  60. ncbi Transgenic cystic fibrosis mice exhibit reduced early clearance of Pseudomonas aeruginosa from the respiratory tract
    T H Schroeder
    Channing Laboratory, Department of Medicine, Brigham and Women s Hospital, Harvard Medical School, Boston, MA 02115, USA
    J Immunol 166:7410-8. 2001
    The cystic fibrosis (CF) transmembrane conductance regulator (CFTR) has been proposed to be an epithelial cell receptor for Pseudomonas aeruginosa involved in bacterial internalization and clearance from the lung...
  61. ncbi Changes in the exocrine pancreas secondary to altered small intestinal function in the CF mouse
    R C De Lisle
    Department of Anatomy and Cell Biology, University of Kansas Medical Center, Kansas City, Kansas 66160, USA
    Am J Physiol Gastrointest Liver Physiol 281:G899-906. 2001
    The exocrine pancreas of the cystic fibrosis (CF) mouse (cftr(m1UNC)) is only mildly affected compared with the human disease, providing a useful model to study alterations in exocrine function...
  62. pmc Intestinal mucins from cystic fibrosis mice show increased fucosylation due to an induced Fucalpha1-2 glycosyltransferase
    Kristina A Thomsson
    Department of Medical Biochemistry, Goteborg University, Medicinaregatan 9A, 41390 Gothenburg, Sweden
    Biochem J 367:609-16. 2002
    ..were purified by three rounds of isopycnic ultracentrifugation from the small and large intestines of CF mice (Cftr(m1UNC)/Cftr(m1UNC)) and compared with wild-type mice...
  63. ncbi Mapping of genetic factors influencing the weight of cystic fibrosis knockout mice
    Christina K Haston
    Program of Genetics and Genomic Biology, The Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada
    Mamm Genome 13:614-8. 2002
    ..Mice in which the CF causative gene, cystic fibrosis transmembrane conductance regulator (Cftr), has been knocked out reflect this as they are smaller than age-matched littermates...
  64. ncbi Residual cftr expression varies with age in cftr(tm1Hgu) cystic fibrosis mice: impact on morphology and physiology
    M Larbig
    Fraunhofer Institute Toxicology and Aerosol Research, Medical School of Hannover, Hannover, Germany
    Pathobiology 70:89-97. 2002
    ..addressed whether aging of the respiratory tract leads to lung pathophysiology in the exon 10 insertional mutant cftr(tm1Hgu) mouse...
  65. pmc HNF1alpha is involved in tissue-specific regulation of CFTR gene expression
    Nathalie Mouchel
    Paediatric Molecular Genetics, Weatherall Institute of Molecular Medicine, Oxford University, John Radcliffe Hospital, Oxford OX3 9DS, UK
    Biochem J 378:909-18. 2004
    The CFTR (cystic fibrosis transmembrane conductance regulator) gene shows a complex pattern of expression with tissue-specific and temporal regulation...
  66. ncbi Inflammation of the cystic fibrosis mouse small intestine
    Oxana Norkina
    Department of Anatomy and Cell Biology, University of Kansas School of Medicine, Kansas City, KS 66160, USA
    Am J Physiol Gastrointest Liver Physiol 286:G1032-41. 2004
    The CFTR null mouse [cystic fibrosis (CF) mouse] has a severe intestinal phenotype that serves as a model for CF-related growth deficiency, meconium ileus, and distal intestinal obstructive syndrome...
  67. pmc Nutritional effects on host response to lung infections with mucoid Pseudomonas aeruginosa in mice
    Anna M van Heeckeren
    Case Western Reserve University School of Medicine, Cleveland, Ohio Hospital San Rafael, La Coruna, Spain
    Infect Immun 72:1479-86. 2004
    In cystic fibrosis, a recessive genetic disease caused by defects in the cystic fibrosis conductance regulator (CFTR), the main cause of death is lung infection and inflammation...
  68. pmc Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis transmembrane regulator knockout murine model
    Peter R Durie
    Program in Integrative Biology, The Research Institute, Toronto, Canada
    Am J Pathol 164:1481-93. 2004
    ..We evaluated multiple organs of congenic C57BL/6J cystic fibrosis transmembrane regulator (Cftr)(-/-) and Cftr(+/+) mice maintained from weaning on a liquid diet then sacrificed between 1 and 24 months of age...
  69. ncbi Acidic duodenal pH alters gene expression in the cystic fibrosis mouse pancreas
    Simran Kaur
    Dept of Anatomy and Cell Biology, Univ of Kansas School of Medicine, Kansas City, KS 66160, USA
    Am J Physiol Gastrointest Liver Physiol 287:G480-90. 2004
    ..acidic in cystic fibrosis (CF) due to decreased bicarbonate ion secretion that is dependent on the CF gene product CFTR. In the CFTR null mouse, the acidic duodenum results in increased signaling from the intestine to the exocrine ..
  70. pmc Instability of the insertional mutation in CftrTgH(neoim)Hgu cystic fibrosis mouse model
    Nikoletta Charizopoulou
    Klinische Forschergruppe, OE 6710, Medizinische Hochschule Hannover, D 30625 Hannover, Germany
    BMC Genet 5:6. 2004
    ..to bypass this time consuming procedure we devised an alternative PCR based protocol whereby mouse strains are differentiated at the Cftr locus by Cftr intragenic microsatellite genotypes that are tightly linked to the disrupted locus.
  71. ncbi Electroneutral sodium absorption and electrogenic anion secretion across murine small intestine are regulated in parallel
    Lara R Gawenis
    Dalton Cardiovascular Research Center, Department of Biomedical Sciences, University of Missouri Columbia, Columbia, MO 65211, USA
    Am J Physiol Gastrointest Liver Physiol 287:G1140-9. 2004
    ..by Na(+)/H(+) exchanger 3 (NHE3) predominates; under stimulated conditions, increased anion secretion mediated by CFTR occurs concurrently with inhibition of Na(+) absorption...
  72. ncbi Targeted inactivation of cystic fibrosis transmembrane conductance regulator chloride channel gene prevents ischemic preconditioning in isolated mouse heart
    Hong Chen
    Department of Pharmacology, Center of Biomedical Research Excellence, University of Nevada School of Medicine, Reno, NV 89557 0270, USA
    Circulation 110:700-4. 2004
    ..In this study, we tested whether the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channels, which are expressed in the heart and activated by protein kinase ..
  73. ncbi Fat absorption in cystic fibrosis mice is impeded by defective lipolysis and post-lipolytic events
    Marcel J C Bijvelds
    Dept of Biochemistry, Erasmus MC, Postbus 1738, NL 3000 DR Rotterdam, The Netherlands
    Am J Physiol Gastrointest Liver Physiol 288:G646-53. 2005
    ..A marked increase in fecal fat excretion was observed in cftr null mice but not in homozygous DeltaF508 mice...
  74. ncbi Down-regulation of the anti-inflammatory protein annexin A1 in cystic fibrosis knock-out mice and patients
    Noura Bensalem
    INSERM U467, Faculte de Medecine Necker, Universite Paris Descartes, France
    Mol Cell Proteomics 4:1591-601. 2005
    ..Because cystic fibrosis (CF) pathophysiology cannot be explained solely by dysfunction of cystic fibrosis transmembrane conductance regulator (CFTR), we applied a proteomic approach (bidimensional electrophoresis and mass ..
  75. ncbi Distinct pattern of lung gene expression in the Cftr-KO mice developing spontaneous lung disease compared with their littermate controls
    Claudine Guilbault
    McGill University Health Center Research Institute, Montreal, Canada
    Physiol Genomics 25:179-93. 2006
    Cystic fibrosis (CF) is caused by a defect in the CF transmembrane conductance regulator (CFTR) protein that functions as a chloride channel...
  76. pmc Mitochondrial oxidative stress in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice
    Leonard W Velsor
    Department of Medicine, National Jewish Medical and Research Center, University of Colorado Health Science Center, Denver, Colorado, USA
    Am J Respir Cell Mol Biol 35:579-86. 2006
    ..fibrosis is a fatal genetic disorder involving dysfunction of the cystic fibrosis transmembrane regulator protein (CFTR) resulting in progressive respiratory failure...
  77. ncbi CFTR regulates phagosome acidification in macrophages and alters bactericidal activity
    Anke Di
    Dept of Neurobiology, Pharmacology and Physiology, University of Chicago, Chicago, IL 60637, USA
    Nat Cell Biol 8:933-44. 2006
    ..Here, we show that in alveolar macrophages the cystic fibrosis transmembrane conductance regulator Cl- channel (CFTR) participates in phagosomal pH control and has bacterial killing capacity...
  78. ncbi Altered transit and bacterial overgrowth in the cystic fibrosis mouse small intestine
    Robert C De Lisle
    Department of Anatomy and Cell Biology, University of Kansas School of Medicine, Kansas City, KS 66160, USA
    Am J Physiol Gastrointest Liver Physiol 293:G104-11. 2007
    ..This work explored two potential factors in development of SIBO in the CF (cftr(tm1UNC)) mouse: impaired Paneth cell innate defenses and altered gastrointestinal motility...
  79. pmc Cystic fibrosis transmembrane conductance regulator is vital to sperm fertilizing capacity and male fertility
    Wen Ming Xu
    Epithelial Cell Biology Research Center, Li Ka Shing Institute of Health Sciences, Department of Physiology, Faculty of Medicine, Chinese University of Hong Kong, Shatin, NT, Hong Kong, China
    Proc Natl Acad Sci U S A 104:9816-21. 2007
    b>Cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel, mutations of which cause cystic fibrosis, a disease characterized by defective Cl(-) and HCO(3)(-) transport...
  80. doi Validation of nasal potential difference measurements in gut-corrected CF knockout mice
    Uta Griesenbach
    Department of Gene Therapy, Faculty of Medicine, National Heart and Lung Institute, Imperial College London, London, United Kingdom
    Am J Respir Cell Mol Biol 39:490-6. 2008
    ..Fatty acid-binding promoter (FABp)-mediated expression of CFTR in the gut, but not the airways, prevents the intestinal disease of the CF knockout mouse...
  81. pmc Role of sphingosine-1-phosphate phosphohydrolase 1 in the regulation of resistance artery tone
    Bernhard Friedrich Peter
    Institute of Physiology, Ludwig Maximilians University, Munich, Germany
    Circ Res 103:315-24. 2008
    ..Inhibition of the cystic fibrosis transmembrane regulator (CFTR) (1) restored S1P responses that were attenuated by SPP1(wt) overexpression; (2) enhanced myogenic vasoconstriction;..
  82. pmc cAMP-mediated regulation of cholesterol accumulation in cystic fibrosis and Niemann-Pick type C cells
    Mary E Manson
    Department of Pediatrics, Case Western Reserve University, 10900 Euclid Ave, Cleveland, OH 44106 4948, USA
    Am J Physiol Lung Cell Mol Physiol 295:L809-19. 2008
    ..Both CFTR activation and expression are regulated by the cAMP pathway, and it is hypothesized that a feedback response ..
  83. doi Azithromycin reduces exaggerated cytokine production by M1 alveolar macrophages in cystic fibrosis
    Magali Meyer
    Department of Clinical Chemistry, Universite Catholique de Louvain, Brussels, Belgium
    Am J Respir Cell Mol Biol 41:590-602. 2009
    ..Azithromycin down-regulates inflammatory cytokine production by M1-polarized CF alveolar macrophages...
  84. pmc Lack of CFTR in skeletal muscle predisposes to muscle wasting and diaphragm muscle pump failure in cystic fibrosis mice
    Maziar Divangahi
    Meakins Christie Laboratories and Respiratory Division, McGill University Health Centre and Research Institute, Montreal, Quebec, Canada
    PLoS Genet 5:e1000586. 2009
    ..Here we show that lack of the CF transmembrane conductance regulator (CFTR) plays an intrinsic role in skeletal muscle atrophy and dysfunction...
  85. pmc Disease-causing mutations in the cystic fibrosis transmembrane conductance regulator determine the functional responses of alveolar macrophages
    Ludmila V Deriy
    Department of Neurobiology, Pharmacology, and Physiology, University of Chicago, Chicago, Illinois 60637, USA
    J Biol Chem 284:35926-38. 2009
    ..Recently, we demonstrated that murine alveolar macrophages employ the cystic fibrosis transmembrane conductance regulator (CFTR) Cl(-) channel as a determinant in lysosomal acidification (Di, A., Brown, M. E...
  86. doi Functional expression of cystic fibrosis transmembrane conductance regulator in mouse chondrocytes
    Haitao Liang
    Transplantation Biology Research Division, State Key Laboratory of Biomembrane and Membrane Biotechnology, Institute of Zoology, Chinese Academy of Sciences, Beijing, China
    Clin Exp Pharmacol Physiol 37:506-8. 2010
    1. Cystic fibrosis transmembrane conductance regulator (CFTR) is well known for its role in the cystic fibrosis (CF)...
  87. pmc The cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in maturation stage ameloblasts, odontoblasts and bone cells
    Antonius Bronckers
    Department of Oral Cell Biology, Academic Centre for Dentistry Amsterdam ACTA, Research Institute MOVE, University of Amsterdam and VU University Amsterdam, The Netherlands
    Bone 46:1188-96. 2010
    Patients with cystic fibrosis (CF) have mild defects in dental enamel. The gene mutated in these patients is CFTR, a Cl(-) channel involved in transepithelial salt and water transport and bicarbonate secretion...
  88. pmc Cardiomyocytes with disrupted CFTR function require CaMKII and Ca(2+)-activated Cl(-) channel activity to maintain contraction rate
    Zachary M Sellers
    Department of Molecular and Integrative Physiology, University of Illinois at Urbana Champaign, Urbana, IL, USA
    J Physiol 588:2417-29. 2010
    The physiological role of the cystic fibrosis transmembrane conductance regulator (CFTR) in cardiomyocytes remains unclear...
  89. pmc A new role for bicarbonate secretion in cervico-uterine mucus release
    Ruth W Muchekehu
    Department of Pediatrics 0830, School of Medicine, University of California San Diego, La Jolla, CA 92093 0830, USA
    J Physiol 588:2329-42. 2010
    ..severely inhibited in the absence of serosal HCO(3)(-), HCO(3)(-) transport, or functional cystic fibrosis transmembrane conductance regulator (CFTR)...
  90. pmc CFTR inhibition provokes an inflammatory response associated with an imbalance of the annexin A1 pathway
    Jesmond Dalli
    The William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, Charterhouse Square, London, EC1M 6BQ, UK
    Am J Pathol 177:176-86. 2010
    Cystic fibrosis (CF), a disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, is characterized by chronic bacterial infections and inflammation in the lung...
  91. doi CXCR2 mediates NADPH oxidase-independent neutrophil extracellular trap formation in cystic fibrosis airway inflammation
    Veronica Marcos
    Research Center, Children s Hospital, Ludwig Maximilians University, Munich, Germany
    Nat Med 16:1018-23. 2010
    ..These studies establish CXCR2 as a receptor mediating NADPH oxidase-independent NET formation and provide evidence that this GPCR pathway is operative and druggable in cystic fibrosis lung disease...
  92. pmc Critical modifier role of membrane-cystic fibrosis transmembrane conductance regulator-dependent ceramide signaling in lung injury and emphysema
    Manish Bodas
    Department of Pediatric Respiratory Sciences, Johns Hopkins University, Baltimore, MD 21287, USA
    J Immunol 186:602-13. 2011
    ..Although an association between lack of cystic fibrosis transmembrane conductance regulator (CFTR) and ceramide accumulation has been described, it is unclear how membrane-CFTR may ..
  93. doi CFTR-deficiency renders mice highly susceptible to cutaneous symptoms during mite infestation
    Yasuaki Hashimoto
    Department of Molecular Medicine, Graduate School of Pharmaceutical Sciences, Global COE Cell Fate Regulation Research and Education Unit, Kumamoto University, Kumamoto, Japan
    Lab Invest 91:509-18. 2011
    ..Here, we identify the cystic fibrosis transmembrane conductance regulator (CFTR/Cftr), an integral membrane protein that mediates transepithelial chloride transport, ..
  94. pmc Listeria monocytogenes exploits cystic fibrosis transmembrane conductance regulator (CFTR) to escape the phagosome
    Andrea L Radtke
    Department of Microbiology and Immunology, University of Michigan Medical School, Ann Arbor, MI 48109, USA
    Proc Natl Acad Sci U S A 108:1633-8. 2011
    ..Here we show that the cystic fibrosis transmembrane conductance regulator (CFTR) facilitates Listeria cytosolic entry...
  95. pmc Role of CFTR expressed by neutrophils in modulating acute lung inflammation and injury in mice
    Xiao Su
    Cardiovascular Research Institute, University of California, San Francisco, HSW 825, 505 Parnassus Ave, San Francisco, CA 94143 0130, USA
    Inflamm Res 60:619-32. 2011
    b>Cystic fibrosis transmembrane conductance regulator (CFTR) regulates infection and inflammation...
  96. doi Mouse models of cystic fibrosis: phenotypic analysis and research applications
    Martina Wilke
    Erasmus MC, Biochemistry Department, Rotterdam, The Netherlands
    J Cyst Fibros 10:S152-71. 2011
    ..Novel data are presented on the intestinal and nasal physiology of F508del-CFTR CF mice backcrossed onto different genetic backgrounds...
  97. pmc Absence of the cystic fibrosis transmembrane regulator (Cftr) from myeloid-derived cells slows resolution of inflammation and infection
    T L Bonfield
    Department of Pediatrics, Case Western Reserve University, Cleveland, OH 44106 4948, USA
    J Leukoc Biol 92:1111-22. 2012
    The absence or reduction of CFTR function causes CF and results in a pulmonary milieu characterized by bacterial colonization and unresolved inflammation...
  98. pmc CFTR regulates early pathogenesis of chronic obstructive lung disease in βENaC-overexpressing mice
    Bjarki Johannesson
    Department of Translational Pulmonology, Translational Lung Research Center Heidelberg, Member of the German Center for Lung Research, University of Heidelberg, Heidelberg, Germany
    PLoS ONE 7:e44059. 2012
    ....
  99. pmc Important role of platelets in modulating endotoxin-induced lung inflammation in CFTR-deficient mice
    Caiqi Zhao
    Key Laboratory of Molecular Virology and Immunology, Institut Pasteur of Shanghai, Chinese Academy of Sciences, Shanghai, China
    PLoS ONE 8:e82683. 2013
    Mutation of CFTR (cystic fibrosis transmembrane conductance regulator) leads to cystic fibrosis (CF). Patients with CF develop abnormalities of blood platelets and recurrent lung inflammation...
  100. ncbi Hyperabsorption of Na+ and raised Ca(2+)-mediated Cl- secretion in nasal epithelia of CF mice
    B R Grubb
    Division of Pulmonary Diseases, University of North Carolina at Chapel Hill 27599
    Am J Physiol 266:C1478-83. 1994
    We investigated the effect of homozygous genetic disruption of the murine cystic fibrosis transmembrane regulator (CFTR) gene on regulation of the rates of Na+ absorption and Cl- secretion by nasal epithelia in cystic fibrosis (CF) mice...
  101. ncbi Long-term survival of the exon 10 insertional cystic fibrosis mutant mouse is a consequence of low level residual wild-type Cftr gene expression
    J R Dorin
    Medical Research Council Human Genetics, Unit, Western General Hospital, Edinburgh, UK
    Mamm Genome 5:465-72. 1994
    ..duplication consequent upon insertional gene targeting allows exon skipping and aberrant splicing to produce normal Cftr mRNA, but at levels greatly reduced compared with wild-type mice...

Research Grants16

  1. Cholesterol transport regulation in cystic fibrosis
    Thomas Kelley; Fiscal Year: 2007
    ..by applicant): The primary cause of cystic fibrosis (CF) is the loss of functional cystic fibrosis transmembrane conductance regulator (CFTR), a cAMP-dependent chloride channel...
  2. TRANSGENIC MOUSE MODELS OF PANCREATIC EXOCRINE FUNCTION
    ROBERT DE LISLE; Fiscal Year: 2003
    ..In CFTR-/- mice, a model for CF, the pancreas exhibits over expression of Muclin, which is associated with protein, plugs ..
  3. PATHOGENEISIS OF CYSTIC FIBROSIS IN THE GI SYSTEM
    ROBERT DE LISLE; Fiscal Year: 2003
    The long-term objective is to understand how loss of functional CFTR (the cystic fibrosis transmembrane regulator) affects expression and post- translational processing of sulfated glycoconjugates and the role of these altered ..
  4. ALTERED EPITHELIAL STAT1 DEPENDENT SIGNALING IN CYSTIC F
    Thomas Kelley; Fiscal Year: 2003
    ..Abstract): Cystic fibrosis (CF) is an inflammatory disease initiated by the loss of cystic fibrosis transmembrane conductance regulator (CFTR) function in epithelial cells...
  5. Redox control of Nrf2/ARE by thioredoxin and GSH
    Jason Hansen; Fiscal Year: 2005
    ..The roles of GSH and cytosolic and nuclear TRX during NRF2 activation and binding will provide an essential foundation for future developmental toxicology. ..
  6. IDENTIFICATION OF BIOCHEMICAL ABNORMALITIES IN PCD CILIA
    LAWRENCE OSTROWSKI; Fiscal Year: 2002
    ..Further studies of the role of these proteins in the assembly and function of cilia may also result in improved treatment for other air way diseases. ..
  7. CHOLESTEROL TRANSPORT IN GALLBLADDER EPITHELIAL CELLS
    Rahul Kuver; Fiscal Year: 2003
    ..3. Compare and contrast the mechanisms of activation of ABCA1 via the cAMP responsive and the LXRalpha/RXR- responsive pathways. ..
  8. MUCIN SECRETION IN COLUMNAR EPITHELIAL CELLS
    Rahul Kuver; Fiscal Year: 2003
    ..Studies by our group and others show a complex relationship between CFTR, the chloride channel defective in CF patients, and the processes by which mucin glyproteins are secreted by ..
  9. A Ciliated Cell-Specific Promoter for Gene Therapy of CF
    LAWRENCE OSTROWSKI; Fiscal Year: 2005
    ..In normal airways, the cystic fibrosis transmembrane conductance regulator (CFTR) protein is expressed primarily at the apical surface of ciliated cells and in the ..
  10. P. aeruginosa lung disease in CF
    Anna Van Heeckeren; Fiscal Year: 2006
    ..Therefore, new treatments must be found. CF is caused by a genetic defect in CFTR, which is a protein expressed in epithelial cells of the lung but also in inflammatory cells...
  11. Role of Oxidatve Stress in Chronic Beryllium Disease
    Brian Day; Fiscal Year: 2006
    ..These studies have the potential to further define the etiology of CBD, risk factors, and suggest novel approaches to prevent and treat this disease. ..
  12. Glutathione transport, oxidative stress and lung injury
    Brian Day; Fiscal Year: 2007
    ..Exciting preliminary studies indicate that the genetic defect in the cystic fibrosis transmembrane conductance regulator (CFTR) produces an antioxidant imbalance by decreasing GSH transport into the pulmonary ..
  13. Conditional Deletion of Dnaic1 as a Model of Primary Ciliary Dyskinesia
    LAWRENCE OSTROWSKI; Fiscal Year: 2007
    ..If successful, this model will also provide a novel system to investigate other questions concerning pulmonary health and disease. [unreadable] [unreadable] [unreadable]..
  14. Biliary oxysterols and cholangiocarcinoma
    RAHUL P KUVER; Fiscal Year: 2010
    ..abstract_text> ..