cystic fibrosis transmembrane conductance regulator

Summary

Gene Symbol: cystic fibrosis transmembrane conductance regulator
Description: cystic fibrosis transmembrane conductance regulator
Alias: RGD1561193, cystic fibrosis transmembrane conductance regulator, ATP-binding cassette sub-family C member 7, ATP-binding cassette transporter sub-family C member 7, cAMP-dependent chloride channel, channel conductance-controlling ATPase, cystic fibrosis transmembrane conductance regulator homolog
Species: rat

Top Publications

  1. 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
  2. ncbi A novel small molecule CFTR inhibitor attenuates HCO3- secretion and duodenal ulcer formation in rats
    Yasutada Akiba
    Department of Medicine, University of California, Los Angeles, USA
    Am J Physiol Gastrointest Liver Physiol 289:G753-9. 2005
  3. doi CFTR inhibition augments NHE3 activity during luminal high CO2 exposure in rat duodenal mucosa
    Misa Mizumori
    Department of Medicine, School of Medicine, University of California Los Angeles, Los Angeles, CA, USA
    Am J Physiol Gastrointest Liver Physiol 294:G1318-27. 2008
  4. pmc Involvement of the cystic fibrosis transmembrane conductance regulator in the acidosis-induced efflux of ATP from rat skeletal muscle
    Jie Tu
    Department of Physiology, Li Ka Shing Faculty of Medicine and Institute of Cardiovascular Science and Medicine, University of Hong Kong, Pokfulam, Hong Kong SAR, China
    J Physiol 588:4563-78. 2010
  5. pmc Syntaxin 3 is necessary for cAMP- and cGMP-regulated exocytosis of CFTR: implications for enterotoxigenic diarrhea
    Anne Collaco
    Department of Pediatrics and Cell Biology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
    Am J Physiol Cell Physiol 299:C1450-60. 2010
  6. pmc Association of a nonsense mutation (W1282X), the most common mutation in the Ashkenazi Jewish cystic fibrosis patients in Israel, with presentation of severe disease
    T Shoshani
    Department of Genetics, Life Sciences Institute, Jerusalem, Israel
    Am J Hum Genet 50:222-8. 1992
  7. ncbi Cystic fibrosis transmembrane conductance regulator protein expression in brain
    A E Mulberg
    Division of Pediatric Gastroenterology and Nutrition, Children s Hospital of Philadelphia, PA
    Neuroreport 5:1684-8. 1994
  8. ncbi A cystic fibrosis mutation associated with mild lung disease
    K H Gan
    Department of Pulmonology, Leyenburg Hospital, The Hague, The Netherlands
    N Engl J Med 333:95-9. 1995
  9. 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
  10. ncbi Mutations in the nucleotide binding domain 1 signature motif region rescue processing and functional defects of cystic fibrosis transmembrane conductance regulator delta f508
    Ana C V DeCarvalho
    Department of Biological Science, Florida State University, Tallahassee, Florida 32306, USA
    J Biol Chem 277:35896-905. 2002

Research Grants

Scientific Experts

Detail Information

Publications101 found, 100 shown here

  1. 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)...
  2. ncbi A novel small molecule CFTR inhibitor attenuates HCO3- secretion and duodenal ulcer formation in rats
    Yasutada Akiba
    Department of Medicine, University of California, Los Angeles, USA
    Am J Physiol Gastrointest Liver Physiol 289:G753-9. 2005
    ..CFTR inhibition reduces acid-induced DBS but also prevents duodenal ulcer formation, supporting our hypothesis that intracellular HCO(3)(-) may be an important protective mechanism for duodenal epithelial cells...
  3. doi CFTR inhibition augments NHE3 activity during luminal high CO2 exposure in rat duodenal mucosa
    Misa Mizumori
    Department of Medicine, School of Medicine, University of California Los Angeles, Los Angeles, CA, USA
    Am J Physiol Gastrointest Liver Physiol 294:G1318-27. 2008
    ..NHE3, by secreting H(+) into the luminal microclimate, facilitates net transmucosal HCO(3)(-) absorption with a mechanism similar to proximal tubular HCO(3)(-) absorption...
  4. pmc Involvement of the cystic fibrosis transmembrane conductance regulator in the acidosis-induced efflux of ATP from rat skeletal muscle
    Jie Tu
    Department of Physiology, Li Ka Shing Faculty of Medicine and Institute of Cardiovascular Science and Medicine, University of Hong Kong, Pokfulam, Hong Kong SAR, China
    J Physiol 588:4563-78. 2010
    ..efflux from the perfused muscle was abolished by CFTR(inh)-172, a specific inhibitor of the cystic fibrosis transmembrane conductance regulator (CFTR), or glibenclamide, an inhibitor of both K(ATP) channels and CFTR, but it was not ..
  5. pmc Syntaxin 3 is necessary for cAMP- and cGMP-regulated exocytosis of CFTR: implications for enterotoxigenic diarrhea
    Anne Collaco
    Department of Pediatrics and Cell Biology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
    Am J Physiol Cell Physiol 299:C1450-60. 2010
    ..cholerae and Escherichia coli cannot elicit fluid secretion in the absence of functional cystic fibrosis transmembrane conductance regulator (CFTR) chloride channels...
  6. pmc Association of a nonsense mutation (W1282X), the most common mutation in the Ashkenazi Jewish cystic fibrosis patients in Israel, with presentation of severe disease
    T Shoshani
    Department of Genetics, Life Sciences Institute, Jerusalem, Israel
    Am J Hum Genet 50:222-8. 1992
    ..In conclusion, the W1282X mutation is the most common cystic fibrosis mutation in the Ashkenazi Jewish patient population in Israel. This nonsense mutation is associated with presentation of severe disease...
  7. ncbi Cystic fibrosis transmembrane conductance regulator protein expression in brain
    A E Mulberg
    Division of Pediatric Gastroenterology and Nutrition, Children s Hospital of Philadelphia, PA
    Neuroreport 5:1684-8. 1994
    The cystic fibrosis transmembrane conductance regulator protein (CFTR) has been identified in bovine brain clathrin-coated vesicles, rat brain and a human neuroblastoma cell line using affinity-purified polyclonal peptide antibodies ..
  8. ncbi A cystic fibrosis mutation associated with mild lung disease
    K H Gan
    Department of Pulmonology, Leyenburg Hospital, The Hague, The Netherlands
    N Engl J Med 333:95-9. 1995
    ..delta F508 is the most common mutation and A455E the second most common mutation of the cystic fibrosis transmembrane conductance regulator gene on chromosome 7...
  9. 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
    ....
  10. ncbi Mutations in the nucleotide binding domain 1 signature motif region rescue processing and functional defects of cystic fibrosis transmembrane conductance regulator delta f508
    Ana C V DeCarvalho
    Department of Biological Science, Florida State University, Tallahassee, Florida 32306, USA
    J Biol Chem 277:35896-905. 2002
    The gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR), an ATP binding cassette (ABC) transporter that functions as a phosphorylation- and nucleotide-regulated chloride channel, is mutated in cystic fibrosis (CF)..
  11. ncbi The cystic fibrosis transmembrane conductance regulator interacts with and regulates the activity of the HCO3- salvage transporter human Na+-HCO3- cotransport isoform 3
    Meeyoung Park
    Department of Physiology, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA
    J Biol Chem 277:50503-9. 2002
    b>Cystic fibrosis transmembrane conductance regulator (CFTR) regulates both HCO(3)(-) secretion and HCO(3)(-) salvage in secretory epithelia...
  12. ncbi Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Asians with chronic pulmonary disease: a pilot study
    Nicola S P Ngiam
    Department of Paediatrics, Yong Loo Lin School of Medicine, National University of Singapore, 5, Lower Kent Ridge Road, Singapore 119074, Singapore
    J Cyst Fibros 5:159-64. 2006
    Little is known about the relationship between cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Asian patients and severe asthma or idiopathic bronchiectasis...
  13. ncbi CFTR genotype as a predictor of prognosis in cystic fibrosis
    Edward F McKone
    Division of Pulmonary and Critical Care Medicine and Adult Cystic Fibrosis Center, University of Washington Medical Center, Seattle, WA 98195 6522, USA
    Chest 130:1441-7. 2006
    ..Certain CFTR genotypes are associated with reduced mortality. The accuracy of using CFTR genotype as a predictor of survival and the mechanisms through which CFTR genotype influences survival are unknown...
  14. 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
    ..These results raise important questions concerning the mechanisms governing tracheal development within the context of CFTR protein dysfunction and the implication of such abnormalities in the pathogenesis of airway disease in CF...
  15. pmc Pulmonary nontuberculous mycobacterial disease: prospective study of a distinct preexisting syndrome
    Richard D Kim
    Immunopathogenesis Section, Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, MD 20892 1684, USA
    Am J Respir Crit Care Med 178:1066-74. 2008
    ..Pulmonary nontuberculous mycobacterial (PNTM) disease is increasing, but predisposing features have been elusive...
  16. ncbi Mutation analysis of the CFTR gene in Slovak cystic fibrosis patients by DHPLC and subsequent sequencing: identification of four novel mutations
    Peter Kolesar
    Department of Molecular Biology, Faculty of Natural Science, Comenius University, Bratislava, Slovakia
    Gen Physiol Biophys 27:299-305. 2008
    ..Four mutations (G437D, H954P, H1375N, and 3120+33G>T) are novel, not yet found in any other CF patient all over the word...
  17. doi The very low penetrance of cystic fibrosis for the R117H mutation: a reappraisal for genetic counselling and newborn screening
    C Thauvin-Robinet
    Centre de Genetique, Hôpital d Enfants, 10, Bd Maréchal de Lattre de Tassigny, BP 77 908, 21079 Dijon Cedex, France
    J Med Genet 46:752-8. 2009
    ..The high frequency of R117H observed in CF newborn screening has also introduced diagnostic dilemmas. The aim of this study was to evaluate the disease penetrance for R117H in order to improve clinical practice...
  18. doi Cystic fibrosis genotype and assessing rates of decline in pulmonary status
    Robert H Cleveland
    Department of Radiology, Division of Respiratory Diseases, Children s Hospital Boston, Harvard Medical School, 300 Longwood Ave, Boston, MA 02115, USA
    Radiology 253:813-21. 2009
    To evaluate the hierarchical phenotypic expression of cystic fibrosis transmembrane conductance regulator (CFTR) genotypes in the respiratory system as has been documented in the pancreas.
  19. pmc Modulation of cystic fibrosis transmembrane conductance regulator (CFTR) activity and genistein binding by cytosolic pH
    Raffaella Melani
    Laboratorio di Genetica Molecolare, Istituto Giannina Gaslini, 16148 Genoa, Italy
    J Biol Chem 285:41591-6. 2010
    Potentiators are molecules that increase the activity of the cystic fibrosis transmembrane conductance regulator (CFTR). Some potentiators can also inhibit CFTR at higher concentrations...
  20. doi The possible additional role of the cystic fibrosis transmembrane regulator to motoneuron inhibition produced by glycine effects
    F R Morales
    Laboratorio de Neurofisiología Celular, Departamento de Fisiologia, Facultad de Medicina, General Flores 2125, Montevideo 11800, Uruguay
    Neuroscience 177:138-47. 2011
    ..The fact that blocking of the CFTR-chloride channel in brain stem motoneurons influences glycinergic inhibition suggests that this channel may play a complementary role in the glycinergic inhibition that occurs during REM sleep...
  21. pmc Small-molecule inhibitors of the cystic fibrosis transmembrane conductance regulator increase pancreatic endocrine cell development in rat and mouse
    S Zertal-Zidani
    INSERM U845, Research Center Growth and Signalling, Faculte de Medecine, Universite Paris Descartes, Sorbonne Paris Cité, Faculty Necker, 156 rue de Vaugirard, Paris, France
    Diabetologia 56:330-9. 2013
    ..The main objective of this work was to discover new drugs that can activate the differentiation of multipotent pancreatic progenitors into endocrine cells...
  22. pmc cAMP/protein kinase A activates cystic fibrosis transmembrane conductance regulator for ATP release from rat skeletal muscle during low pH or contractions
    Jie Tu
    Department of Physiology, The University of Hong Kong, Pokfulam, Hong Kong, Special Administrative Region, People s Republic of China
    PLoS ONE 7:e50157. 2012
    We have shown that cystic fibrosis transmembrane conductance regulator (CFTR) is involved in ATP release from skeletal muscle at low pH...
  23. pmc Progesterone downregulates oestrogen-induced expression of CFTR and SLC26A6 proteins and mRNA in rats' uteri
    K Gholami
    Department of Molecular Medicine, Faculty of Medicine, University of Malaya, 50603 Kuala Lumpur, Malaysia
    J Biomed Biotechnol 2012:596084. 2012
    ..Under progesterone (P) dominance, fluid loss assists uterine closure which is associated with pH reduction. We hypothesize that P inhibits uterine fluid secretion and HCO3⁻ transport...
  24. ncbi [Effects of weile powder on bicarbonate transporters CFTR SLC26A3 and SLC26A6 in gastric ulcers of rats]
    Guo Rong Wen
    Department of Gastroenterology, Affiliated Hospital, Zunyi Medical College, Guiyang 563000, China
    Zhongguo Zhong Xi Yi Jie He Za Zhi 33:81-4. 2013
    ..To investigate the effects of Weile Powder (WLP) on bicarbonate transporters in rats with gastric ulcers, and to probe its functional mechanisms...
  25. doi Cystic fibrosis transmembrane conductance regulator in the endolymphatic sac of the rat
    Ai Matsubara
    Department of Otolaryngology, Faculty of Medicine, Kagawa University, Japan Electronic address
    Auris Nasus Larynx 41:409-12. 2014
    ..In this study, we confirmed the expression of cystic fibrosis transmembrane conductance regulator (CFTR) in the endolymphatic sac by reverse transcriptase polymerase chain reaction (RT-PCR) ..
  26. pmc Characterization of defects in ion transport and tissue development in cystic fibrosis transmembrane conductance regulator (CFTR)-knockout rats
    Katherine L Tuggle
    Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, Alabama, United States of America Department of Environmental Health Sciences, School of Public Health, University of Alabama at Birmingham, Birmingham, Alabama, United States of America
    PLoS ONE 9:e91253. 2014
    ..we describe development and characterization of the first cystic fibrosis rat, in which the cystic fibrosis transmembrane conductance regulator gene (CFTR) was knocked out using a pair of zinc finger endonucleases (ZFN)...
  27. doi Aquaporin-4 as a molecular partner of cystic fibrosis transmembrane conductance regulator in rat Sertoli cells
    Tito T Jesus
    CICS UBI Health Sciences Research Centre, University of Beira Interior, Covilha, Portugal
    Biochem Biophys Res Commun 446:1017-21. 2014
    ..The cystic fibrosis transmembrane conductance regulator (CFTR) plays an essential role to male fertility and it was recently suggested that it may ..
  28. doi Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) prevents apoptosis induced by hydrogen peroxide in basilar artery smooth muscle cells
    Jia Wei Zeng
    Department of Pharmacology, and Cardiac and Cerebral Vascular Research Center, Zhongshan School of Medicine, Sun Yat Sen University, Guangzhou, 510080, People s Republic of China
    Apoptosis 19:1317-29. 2014
    b>Cystic fibrosis transmembrane conductance regulator (CFTR) acts as a cAMP-dependent chloride channel, has been studied in various types of cells...
  29. pmc Bioactivity-guided fractionation of an antidiarrheal Chinese herb Rhodiola kirilowii (Regel) Maxim reveals (-)-epicatechin-3-gallate and (-)-epigallocatechin-3-gallate as inhibitors of cystic fibrosis transmembrane conductance regulator
    Lei Chen
    School of Life Sciences, Liaoning Provincial Key Laboratory of Biotechnology and Drug Discovery, Liaoning Normal University, Dalian, 116029, P R China
    PLoS ONE 10:e0119122. 2015
    b>Cystic fibrosis transmembrane conductance regulator (CFTR) is the principal apical route for transepithelial fluid transport induced by enterotoxin...
  30. pmc Functional characteristics of L1156F-CFTR associated with alcoholic chronic pancreatitis in Japanese
    Shiho Kondo
    Department of Human Nutrition, Nagoya University Graduate School of Medicine, Nagoya, Japan
    Am J Physiol Gastrointest Liver Physiol 309:G260-9. 2015
    ..fibrosis is rare in Japanese, measurement of sweat Cl(-) has suggested mild dysfunction of cystic fibrosis transmembrane conductance regulator (CFTR) in some patients with chronic pancreatitis...
  31. doi Ivacaftor potentiation of multiple CFTR channels with gating mutations
    Haihui Yu
    Vertex Pharmaceuticals Incorporated, Cambridge, MA 02139, USA
    J Cyst Fibros 11:237-45. 2012
    ..The aim of this in vitro study was to determine whether ivacaftor potentiates mutant CFTR with gating defects caused by other CFTR gating mutations...
  32. doi Up-regulated cystic fibrosis transmembrane conductance regulator after anal stenosis in rats
    Jonathan Ramprasad
    Department of Pediatric Gastroenterology, Hepatology and Nutrition, Medical College of Wisconsin, Milwaukee, WI 53295, USA
    J Pediatr Surg 47:556-64. 2012
    ..We hypothesize that fecal impaction elicits up-regulation of cystic fibrosis transmembrane conductance regulator (CFTR), a cAMP-activated mucosal chloride channel.
  33. doi Cryptorchidism-induced CFTR down-regulation results in disruption of testicular tight junctions through up-regulation of NF-κB/COX-2/PGE2
    Jing Chen
    Epithelial Cell Biology Research Center, School of Biomedical Sciences, Faculty of Medicine, The Chinese University of Hong Kong, Shatin, NT, Hong Kong
    Hum Reprod 27:2585-97. 2012
    Does elevated temperature-induced cystic fibrosis transmembrane conductance regulator (CFTR) down-regulation in Sertoli cells in cryptorchid testis disrupt testicular tight junctions (TJs) through the nuclear factor kappa B (NF-κB)/..
  34. doi Protective role of cardiac CFTR activation upon early reperfusion against myocardial infarction
    Hiromi Uramoto
    Department of Cell Physiology, National Institute for Physiological Sciences, Myodaiji cho, Okazaki, Japan
    Cell Physiol Biochem 30:1023-38. 2012
    The cardiac isoform of the cystic fibrosis transmembrane conductance regulator (CFTR) was shown to be activated by β-adrenergic or purinergic stimulation and involved in cell volume regulation after osmotic swelling...
  35. ncbi A cystic fibrosis allele encoding missense mutations in both nucleotide binding folds of the cystic fibrosis transmembrane conductance regulator
    N Kalin
    Abteilung Biophysikalische Chemie, Medizinische Hochschule Hannover, Germany
    Hum Mutat 1:204-10. 1992
    ..German cystic fibrosis (CF) chromosomes were screened for molecular lesions in exon 20 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene by chemical cleavage of mismatch...
  36. pmc Cystic fibrosis patients bearing both the common missense mutation Gly----Asp at codon 551 and the delta F508 mutation are clinically indistinguishable from delta F508 homozygotes, except for decreased risk of meconium ileus
    A Hamosh
    Center for Medical Genetics, School of Hygiene and Public Health, Johns Hopkins University, Baltimore, MD
    Am J Hum Genet 51:245-50. 1992
    ..mutation at codon 551 (G551D), which is within the first nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator (CFTR), is the third most common cystic fibrosis (CF) mutation, with a worldwide frequency ..
  37. pmc Nine cystic fibrosis patients homozygous for the CFTR nonsense mutation R1162X have mild or moderate lung disease
    P Gasparini
    Institute of Biological Sciences, University of Verona, Italy
    J Med Genet 29:558-62. 1992
    ..These results suggest that this form of truncated CFTR protein, still containing the regulatory region, the first ATP binding domain, and both transmembrane domains, could be partially working in the lung tissues...
  38. ncbi Multiple mutations in highly conserved residues are found in mildly affected cystic fibrosis patients
    M Dean
    Biological Carcinogenesis and Development Program, Program Resources, Inc, Frederick, Maryland
    Cell 61:863-70. 1990
    We have identified three different point mutations in the coding region of the cystic fibrosis transmembrane conductance regulator (CFTR) gene...
  39. ncbi A novel mutation in the cystic fibrosis gene in patients with pulmonary disease but normal sweat chloride concentrations
    W E Highsmith
    Applied Technology Genetics Corp, Malvern, PA
    N Engl J Med 331:974-80. 1994
    ..It has been difficult to make the diagnosis of cystic fibrosis in these patients because no associated mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene has been identified.
  40. ncbi Analysis of the mouse and rat CFTR promoter regions
    E Denamur
    Department of Laboratory Medicine, University of California, San Francisco 94143 0134
    Hum Mol Genet 3:1089-94. 1994
    To gain insights into the regulation of the mouse and rat cystic fibrosis transmembrane conductance regulator (CFTR) genes, we cloned and sequenced their respective upstream promoter regions...
  41. ncbi Increased incidence of cystic fibrosis gene mutations in adults with disseminated bronchiectasis
    P F Pignatti
    Institute of Biology and Genetics, University of Verona School of Medicine, Italy
    Hum Mol Genet 4:635-9. 1995
    ..These results indicate that CFTR gene mutations, and perhaps also DNA polymorphisms, may be involved in the etiopathogenesis of at least some cases of bronchiectasis...
  42. 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)...
  43. ncbi Phylogenetic analysis of cystic fibrosis transmembrane conductance regulator gene in mammalian species argues for the development of a rabbit model for cystic fibrosis
    S Vuillaumier
    INSERM U120, Hopital Robert Debre, Paris, France
    Mol Biol Evol 14:372-80. 1997
    The species-specific pattern of cystic fibrosis transmembrane conductance regulator (CFTR) expression was investigated in order to identify species closely related to man which can be used as potential cystic fibrosis (CF) animal models...
  44. ncbi Distinct spectrum of CFTR gene mutations in congenital absence of vas deferens
    T Dork
    Institut fur Humangenetik, Medizinische Hochschule Hannover, Germany
    Hum Genet 100:365-77. 1997
    ..A high incidence of mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene has recently been reported in males with CAVD...
  45. ncbi Detection of CFTR gene mutations in patients suffering from chronic bronchitis
    M Kostuch
    Department of Human Genetics, Lublin School of Medicine, Lublin, Poland
    Arch Med Res 31:97-100. 2000
    The purpose of the study was to examine cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in patients suffering from chronic bronchitis.
  46. ncbi Subcellular distribution of CFTR in rat intestine supports a physiologic role for CFTR regulation by vesicle traffic
    N A Ameen
    Department of Pediatrics, University of Miami School of Medicine, FL 33136, USA
    Histochem Cell Biol 114:219-28. 2000
    The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-activated chloride channel critical to intestinal anion secretion. In addition to phosphorylation, vesicle traffic regulates CFTR in some epithelial cells...
  47. ncbi Frequency of cystic fibrosis transmembrane conductance regulator gene mutations and 5T allele in patients with allergic bronchopulmonary aspergillosis
    E Marchand
    Service de Pneumologie, Cliniques Universitaires de Mont Godinne, Universite Catholique de Louvain, Yvoir, Belgium
    Chest 119:762-7. 2001
    To assess the frequency of cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in patients with allergic bronchopulmonary aspergillosis (ABPA).
  48. ncbi G551D CF mice display an abnormal host response and have impaired clearance of Pseudomonas lung disease
    B J McMorran
    Institute for Molecular Biosciences, University of Queensland, St Lucia, Queensland 4072, Australia
    Am J Physiol Lung Cell Mol Physiol 281:L740-7. 2001
    ....
  49. ncbi Independent amplification of two gene clusters on chromosome 4 in rat endometrial cancer: identification and molecular characterization
    A Walentinsson
    Department of Cell and Molecular Biology Genetics, Goteborg University, SE 40530 Gothenburg, Sweden
    Cancer Res 61:8263-73. 2001
    ..Taken together, our findings suggest that up-regulation of Cdk6 and/or Met may contribute to the development of endometrial cancers in the BDII rat...
  50. ncbi Increased prevalence of mutations in the cystic fibrosis transmembrane conductance regulator in children with chronic rhinosinusitis
    Vidya Raman
    Department of Pediatrics, Washington University School of Medicine and St Louis Children s Hospital, St Louis, Missouri, USA
    Pediatrics 109:E13. 2002
    ..Our objective was to study the frequency of CFTR mutations among children with chronic rhinosinusitis...
  51. ncbi Reciprocal protein kinase A regulatory interactions between cystic fibrosis transmembrane conductance regulator and Na+/H+ exchanger isoform 3 in a renal polarized epithelial cell model
    Anna Bagorda
    Department of General and Environmental Physiology, University of Bari, Bari 70126, Italy
    J Biol Chem 277:21480-8. 2002
    Although Cystic fibrosis transmembrane conductance regulator (CFTR) has been shown to regulate the activity of NHE3, the potential reciprocal interaction of NHE3 to modulate the protein kinase A (PKA)-dependent regulation of CFTR in ..
  52. ncbi Quantification of CFTR splice variants in adults with disseminated bronchiectasis, using the TaqMan fluorogenic detection system
    J Andrieux
    Laboratoire de Genetique Moleculaire, EFS Bretagne CHU, 29200 Brest, France
    Clin Genet 62:60-7. 2002
    Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene are associated with different related disorders such as congenital bilateral absence of the vas deferens, chronic idiopathic pancreatitis, or disseminated ..
  53. ncbi Ischemia-induced enhancement of CFTR expression on the plasma membrane in neonatal rat ventricular myocytes
    Hiromi Uramoto
    Department of Cell Physiology, National Institute for Physiological Sciences and Japan Science and Technology Agency, Okazaki, 444 8585 Japan
    Jpn J Physiol 53:357-65. 2003
    Pathophysiological functions of cardiac cystic fibrosis transmembrane conductance regulator (cCFTR) in ischemia are not well known...
  54. ncbi Regulation of the cystic fibrosis transmembrane conductance regulator channel by beta-adrenergic agonists and vasoactive intestinal peptide in rat smooth muscle cells and its role in vasorelaxation
    Renaud Robert
    Laboratoire des Biomembranes et Signalisation Cellulaire CNRS Unité Mixte de Recherche 6558, Universite de Poitiers, 86022 Poitiers, France
    J Biol Chem 279:21160-8. 2004
    ..Here we show expression of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl(-) channel in rat aortic smooth muscle cells...
  55. ncbi Evidence of a functional CFTR Cl(-) channel in adult alveolar epithelial cells
    Emmanuelle Brochiero
    Départemente de Médecine, Universite de Montreal, Montresl, Quebec, Canada H2W 1T7
    Am J Physiol Lung Cell Mol Physiol 287:L382-92. 2004
    The cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in the fetal lung, but during lung development it gradually disappears in cells of future alveolar spaces...
  56. ncbi High frequency of the R75Q CFTR variation in patients with chronic obstructive pulmonary disease
    Aleksandra Divac
    Institute of Molecular Genetics and Genetic Engineering, Vojvode Stepe 444a, 11000 Belgrade, Serbia and Montenegro
    J Cyst Fibros 3:189-91. 2004
    ..002). In all patients carrying the R75Q chronic bronchitis was a dominant symptom of COPD, and all were homozygous for the V470 allele. These findings suggest that R75Q mutation could be characteristic CFTR variant for COPD patients...
  57. ncbi Increased expression of cystic fibrosis transmembrane conductance regulator in rat liver after common bile duct ligation
    Hong Shen
    Faculty of Pharmacy, University of Manitoba, Winnipeg, Manitoba, Canada
    J Cell Physiol 203:599-603. 2005
    ..The autosomal-recessive disorder of CF results from mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) that encodes the CFTR protein...
  58. pmc Pathophysiologic consequences following inhibition of a CFTR-dependent developmental cascade in the lung
    J Craig Cohen
    Louisiana State University Health Sciences Center, Department of Medicine, School of Medicine, New Orleans, LA 70112, USA
    BMC Dev Biol 5:2. 2005
    ..This problem is particularly apparent in evaluating the developmental role of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in the cystic fibrosis (CF) phenotype...
  59. ncbi Increased prevalence of chronic rhinosinusitis in carriers of a cystic fibrosis mutation
    Xinjing Wang
    McKusick Nathans Institute of Genetic Medicine, Johns Hopkins University, Baltimore, MD, USA
    Arch Otolaryngol Head Neck Surg 131:237-40. 2005
    ..Cystic fibrosis is an inherited recessive disorder that invariably affects the sinuses. The frequency of CF mutations has been reported to be higher in patients with CRS than in unaffected controls...
  60. pmc Cystic fibrosis transmembrane regulator gene carrier status is a risk factor for young onset pancreatic adenocarcinoma
    R McWilliams
    Gut 54:1661-2. 2005
  61. ncbi Differential sensitivity of the cystic fibrosis (CF)-associated mutants G551D and G1349D to potentiators of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel
    Zhiwei Cai
    Department of Physiology, University of Bristol, School of Medical Sciences, University Walk, Bristol BS8 1TD, United Kingdom
    J Biol Chem 281:1970-7. 2006
    The genetic disease cystic fibrosis (CF) is caused by loss of function of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel...
  62. ncbi Bicarbonate secretion by rat bile duct brush cells indicated by immunohistochemical localization of CFTR, anion exchanger AE2, Na+/HCO3 -cotransporter, carbonic anhydrase II, Na+/H+ exchangers NHE1 and NHE3, H+/K+-ATPase, and Na+/K+-ATPase
    Takuro Ogata
    Department of Surgery and Physiology, Kochi Medical School, Kohasu, Nankoku, Japan
    Med Mol Morphol 39:44-8. 2006
    ..Immunoreactivity of proteins involved in Cl(-)/HCO(3) (-) exchange reaction, cystic fibrosis transmembrane conductance regulator (CFTR) and Cl(-)/HCO(3) (-) exchanger (AE2), was found on the microvilli (MV) and along the ..
  63. ncbi Atrial natriuretic peptide modulates cystic fibrosis transmembrane conductance regulator chloride channel expression in rat proximal colon and human intestinal epithelial cells
    H J Novaira
    Instituto de Biofisica Carlos Chagas Filho, Universidade Federal do Rio de Janeiro, CCS Bloco G, 21949 900 Rio de Janeiro, Brazil
    J Endocrinol 189:155-65. 2006
    The cystic fibrosis transmembrane conductance regulator (CFTR) is one of the most intensively investigated Cl- channels. Different mutations in the CFTR gene cause the disease cystic fibrosis (CF)...
  64. ncbi Cystic fibrosis transmembrane conductance regulator deficiency exacerbates islet cell dysfunction after beta-cell injury
    Michael S Stalvey
    Department of Pathology, University of Florida, College of Medicine, PO Box 100275, Gainesville, FL 32610, USA
    Diabetes 55:1939-45. 2006
    The cause of cystic fibrosis-related diabetes (CFRD) remains unknown, but cystic fibrosis transmembrane conductance regulator (CFTR) mutations contribute directly to multiple aspects of the cystic fibrosis phenotype...
  65. ncbi Denopamine stimulates alveolar fluid clearance via cystic fibrosis transmembrane conductance regulator in rat lungs
    Xiu Gu
    Thoracic Surgery, Kanazawa Medical University, Uchinada, Ishikawa 920 0293, Japan
    Respirology 11:566-71. 2006
    The objective of this study was to test the hypothesis that cystic fibrosis transmembrane conductance regulator (CFTR) plays a role in beta(1)-adrenergic agonist-stimulated alveolar fluid clearance.
  66. ncbi WNK1 and WNK4 modulate CFTR activity
    Chao Ling Yang
    Division of Nephrology and Hypertension, Department of Medicine, Oregon Health and Science University, Portland, OR 97239, USA
    Biochem Biophys Res Commun 353:535-40. 2007
    The cystic fibrosis transmembrane conductance regulator (CFTR) is an ATP-gated chloride channel. WNK kinases are widely expressed modulators of ion transport...
  67. ncbi Interplay between cystic fibrosis transmembrane regulator and gap junction channels made of connexins 45, 40, 32 and 50 expressed in oocytes
    Basilio A Kotsias
    Department of Pharmacology and Physiology, University of Rochester School of Medicine, 601 Elmwood Avenue, Rochester, NY 14642 8711, USA
    J Membr Biol 214:1-8. 2006
    ..The data suggest a cross-talk between CFTR and a variety of gap junction channels. Cytoskeletal scaffolding proteins and/or other intermediate cytoplasmic proteins are likely to play a role in CFTR-Cx interaction...
  68. ncbi Thyroid hormones stimulate renal expression of CFTR
    Ana C O de Andrade Pinto
    Instituto de Biofisica Carlos Chagas Filho, Universidade Federal do Rio de Janeiro, Brazil
    Cell Physiol Biochem 20:83-90. 2007
    ..05). Analysis of the promoter region of CFTR transfected to IRPTC showed that T(3) (10(-7) M) stimulates the CFTR promoter (38%, n=4, p<0.05)...
  69. ncbi Expression and function of cystic fibrosis transmembrane conductance regulator in rat intrapulmonary arteries
    R Robert
    Institute of Cellular Physiology and Biology, UMR 6187, National Centre for Scientific Research, University of Poitiers, Poitiers, France
    Eur Respir J 30:857-64. 2007
    The cystic fibrosis transmembrane conductance regulator (CFTR) gene encodes a cyclic adenosine monophosphate (cAMP)-dependent chloride channel located mainly at the apical membrane of epithelial cells...
  70. pmc Cyclic AMP regulates bicarbonate secretion in cholangiocytes through release of ATP into bile
    Noritaka Minagawa
    Department of Medicine, Yale University School of Medicine, New Haven, Connecticut, USA
    Gastroenterology 133:1592-602. 2007
    ..We examined the role of the inositol 1,4,5-trisphosphate receptor (InsP3R) in mediating bicarbonate secretion because this is the only intracellular Ca(2+) release channel in cholangiocytes...
  71. ncbi Adenosine receptors in rat and human pancreatic ducts stimulate chloride transport
    Ivana Novak
    Department of Biosciences, August Krogh Building, University of Copenhagen, Universitetsparken 13, 2100 Copenhagen, Denmark
    Pflugers Arch 456:437-47. 2008
    ..We propose that adenosine can stimulate pancreatic secretion and, thereby, is an active player in the acini-to-duct signaling...
  72. ncbi Immunohistochemical localization of cystic fibrosis transmembrane regulator and clara cell secretory protein in taste receptor cells of rat circumvallate papillae
    Flavia Merigo
    Human Anatomy and Histology Section, Morphological Biomedical Sciences Department, University of Verona, Strada Le Grazie 8, I 37134 Verona, Italy
    Chem Senses 33:231-41. 2008
    ....
  73. pmc RNA interference for CFTR attenuates lung fluid absorption at birth in rats
    Tianbo Li
    Department of Integrative Medical Sciences, Northeastern Ohio Universities Colleges of Medicine and Pharmacy, Rootstown, OH 44272 0095, USA
    Respir Res 9:55. 2008
    Small interfering RNA (siRNA) against alphaENaC (alpha-subunit of the epithelial Na channel) and CFTR (cystic fibrosis transmembrane conductance regulator) was used to explore ENaC and CTFR function in newborn rat lungs.
  74. pmc Small nuclear RNAs U11 and U12 modulate expression of TNR-CFTR mRNA in mammalian kidneys
    Jackson Souza-Menezes
    Instituto de Biofisica Carlos Chagas Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil
    Cell Physiol Biochem 22:93-100. 2008
    TNR-CFTR, discovered as a splice variant of CFTR (Cystic Fibrosis Transmembrane conductance Regulator), is distributed in different tissues such as human and rat kidney, trachea, lungs etc and is a functional chloride channel...
  75. ncbi Functional expression of cystic fibrosis transmembrane conductance regulator in rat oviduct epithelium
    Minhui Chen
    School of Life Science, Sun Yat Sen University, Guangzhou 510275, China
    Acta Biochim Biophys Sin (Shanghai) 40:864-72. 2008
    The aim of this study was to investigate the functional expression of cystic fibrosis transmembrane conductance regulator (CFTR) with electrophysiological and molecular technique in rat oviduct epithelium...
  76. pmc Hepatic cystogenesis is associated with abnormal expression and location of ion transporters and water channels in an animal model of autosomal recessive polycystic kidney disease
    Jesus M Banales
    Miles and Shirley Fiterman Center for Digestive Diseases, Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA
    Am J Pathol 173:1637-46. 2008
    ..Therapeutic interventions that block the activation of these proteins might inhibit cyst expansion in polycystic liver disease...
  77. pmc Rab11b regulates the apical recycling of the cystic fibrosis transmembrane conductance regulator in polarized intestinal epithelial cells
    Mark R Silvis
    Department of Cell Biology and Physiology, University of Pittsburgh School of Medicine, PA 15261, USA
    Mol Biol Cell 20:2337-50. 2009
    The cystic fibrosis transmembrane conductance regulator (CFTR), a cAMP/PKA-activated anion channel, undergoes efficient apical recycling in polarized epithelia...
  78. pmc A truncated CFTR protein rescues endogenous DeltaF508-CFTR and corrects chloride transport in mice
    Estelle Cormet-Boyaka
    Division of Pulmonary Critical Care and Sleep Medicine, The Ohio State University, Columbus, OH 43201, USA
    FASEB J 23:3743-51. 2009
    ..These findings identify complementary protein fragments as a viable in vivo approach for correcting disease-causing misfolding of plasma membrane proteins...
  79. pmc Lubiprostone stimulates duodenal bicarbonate secretion in rats
    Misa Mizumori
    Department of Medicine, University of California Los Angeles, Los Angeles, CA, USA
    Dig Dis Sci 54:2063-9. 2009
    ..Lubiprostone, a bicyclic fatty acid, is used for the treatment of chronic constipation. No published study has addressed the effect of lubiprostone on intestinal ion secretion in vivo...
  80. pmc Characteristics of Cl- uptake in rat alveolar type I cells
    Meshell Johnson
    Department of Medicine, University of California, San Francisco, 3333 California St, Suite 150, Box 1245, San Francisco, CA 94118, USA
    Am J Physiol Lung Cell Mol Physiol 297:L816-27. 2009
    ....
  81. doi Non-classic cystic fibrosis associated with D1152H CFTR mutation
    P R Burgel
    Hopital Cochin, APHP, Universite Paris Descartes, Paris, France
    Clin Genet 77:355-64. 2010
    Limited knowledge exists on phenotypes associated with the D1152H cystic fibrosis transmembrane conductance regulator (CFTR) mutation.
  82. pmc Familial clustering of pulmonary nontuberculous mycobacterial disease
    Rhonda E Colombo
    Immunopathogenesis Section, Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, Clinical Center, National Institutes of Health, Bethesda, MD 20892 1888, USA
    Chest 137:629-34. 2010
    ..Although genetic causes of disseminated NTM infection are well characterized, genetic causes for most human susceptibility to pulmonary NTM infection have not been determined...
  83. pmc Genomewide association analysis of respiratory syncytial virus infection in mice
    James M Stark
    Department of Pediatrics, University of Texas Health Science Center, Houston, Texas, USA
    J Virol 84:2257-69. 2010
    ..In addition, we have reported that gene-targeted mice lacking the cystic fibrosis transmembrane conductance regulator (Cftr; ATP-binding cassette subfamily C, member 7) are susceptible to RSV infection...
  84. doi Genetic and biochemical markers of obstructive lung disease in the general population
    Morten Dahl
    Department of Clinical Biochemistry, Herlev Hospital, Copenhagen University Hospital, Faculty of Health Sciences, Herlev, Denmark
    Clin Respir J 3:121-2. 2009
    ..New markers of early disease activity and prognosis are needed in asthma and chronic obstructive pulmonary disease (COPD)...
  85. pmc Alpha-AP-2 directs myosin VI-dependent endocytosis of cystic fibrosis transmembrane conductance regulator chloride channels in the intestine
    Anne Collaco
    Department of Pediatrics and Cell Biology University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15206, USA
    J Biol Chem 285:17177-87. 2010
    The actin motor myosin VI regulates endocytosis of cystic fibrosis transmembrane conductance regulator (CFTR) in the intestine, but the endocytic adaptor linking CFTR to myosin VI is unknown...
  86. doi Cystic fibrosis conductance regulator, tumor necrosis factor, interferon alpha-10, interferon alpha-17, and interferon gamma genotyping as potential risk markers in pulmonary sarcoidosis pathogenesis in Greek patients
    Periklis Makrythanasis
    Department of Medical Genetics, Medical School, University of Athens, Athens, Greece
    Genet Test Mol Biomarkers 14:577-84. 2010
    ....
  87. doi Role of CFTR and ClC-5 in modulating vacuolar H+-ATPase activity in kidney proximal tubule
    Luciene R Carraro-Lacroix
    Department of Physiology and Biophysics, University of Sao Paulo, Sao Paulo, Brazil
    Cell Physiol Biochem 26:563-76. 2010
    ..The present work was performed to study the modulation of plasma membrane H(+)-ATPase by chloride channels, in particular, CFTR and ClC-5 in kidney proximal tubule...
  88. pmc A host defense mechanism involving CFTR-mediated bicarbonate secretion in bacterial prostatitis
    Chen Xie
    Epithelial Cell Biology Research Center, School of Biomedical Sciences, Faculty of Medicine, Chinese University of Hong Kong, Hong Kong, China
    PLoS ONE 5:e15255. 2010
    ..Prostatitis is associated with a characteristic increase in prostatic fluid pH; however, the underlying mechanism and its physiological significance have not been elucidated...
  89. doi Cystic fibrosis transmembrane conductance regulator modulates synaptic chloride homeostasis in motoneurons of the rat spinal cord during neonatal development
    Alexey Ostroumov
    Neurobiology Sector, International School for Advanced Studies SISSA, via Bonomea, Trieste 34136, Italy
    Dev Neurobiol 71:253-68. 2011
    b>Cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-regulated Cl(-) channel functional in neonatal rat spinal motoneurons...
  90. pmc Defective CFTR-dependent CREB activation results in impaired spermatogenesis and azoospermia
    Wen Ming Xu
    Sichuan University The Chinese University of Hong Kong Joint Laboratory for Reproductive Medicine, West China Institute of Women and Children s Health, West China Second University Hospital, Sichuan University, Chengdu, People s Republic of China
    PLoS ONE 6:e19120. 2011
    ..common life-limiting recessive genetic disease among Caucasians caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) with over 95% male patients infertile...
  91. ncbi Localization of the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) in the rat to chromosome 4 and implications for the evolution of mammalian chromosomes
    A E Trezise
    Department of Genetics, Hospital for Sick Children, Toronto, Ontario, Canada
    Genomics 14:869-74. 1992
    We have isolated a partial cDNA encoding the cystic fibrosis transmembrane conductance regulator (CFTR) in the rat. This cDNA hybridizes to a 6...
  92. ncbi Inhibitory regulation of cystic fibrosis transmembrane conductance regulator anion-transporting activities by Shank2
    Joo Young Kim
    Department of Pharmacology and Brain Korea 21 Project fo Medical Science, Yonsei University College of Medicine, Seoul 120 752
    J Biol Chem 279:10389-96. 2004
    ..In the present study, we report on the biochemical and functional association between cystic fibrosis transmembrane conductance regulator (CFTR) and a PDZ domain-containing protein Shank2...
  93. ncbi STa and cGMP stimulate CFTR translocation to the surface of villus enterocytes in rat jejunum and is regulated by protein kinase G
    Franca Golin-Bisello
    Dept of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA 15261, USA
    Am J Physiol Cell Physiol 289:C708-16. 2005
    The cystic fibrosis transmembrane conductance regulator (CFTR) is critical to cAMP- and cGMP-activated intestinal anion secretion and the pathogenesis of secretory diarrhea...
  94. ncbi Estrogen-induced abnormally high cystic fibrosis transmembrane conductance regulator expression results in ovarian hyperstimulation syndrome
    Louis Chukwuemeka Ajonuma
    Epithelial Cell Biology Research Center, Department of Physiology, Faculty of Medicine, Chinese University of Hong Kong, Shatin, New Territory, Hong Kong
    Mol Endocrinol 19:3038-44. 2005
    ..Using RT-PCR, Western blot, and electrophysiological techniques we show that cystic fibrosis transmembrane conductance regulator (CFTR), a cAMP-activated chloride channel expressed in many epithelia, is involved in the ..
  95. ncbi Differential regulation of cystic fibrosis transmembrane conductance regulator by interferon gamma in mast cells and epithelial cells
    Marianna Kulka
    Pulmonary Research Group, University of Alberta, Edmonton, Canada
    J Pharmacol Exp Ther 315:563-70. 2005
    b>Cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-dependent chloride channel in epithelial cells; recently, we identified it in mast cells...
  96. ncbi Cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel and Na-K-Cl cotransporter NKCC1 isoform mediate the vasorelaxant action of genistein in isolated rat aorta
    Marta S Valero
    Department of Physiology and Pharmacology, School of Medicine, University of Zaragoza, Spain
    Eur J Pharmacol 544:126-31. 2006
    ..endothelium-denuded rat aorta to investigate the role of the cyclic AMP(cAMP)-activated, cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel, and its associated Na-K-Cl cotransporter NKCC1...
  97. ncbi Roles of CLCA and CFTR in electrolyte re-absorption from rat saliva
    K Ishibashi
    Department of Functional Bioscience, Fukuoka Dental College, Japan
    J Dent Res 85:1101-5. 2006
    ..To address the question as to whether rCLCA and cystic fibrosis transmembrane conductance regulator (CFTR) are involved in Cl- re-absorption, we evaluated the electrolyte content of saliva ..
  98. ncbi Expression and localization of cystic fibrosis transmembrane conductance regulator in the rat endocrine pancreas
    Alain Boom
    Laboratory of Histology, Neuroanatomy and Neuropathology, School of Medicine, Universite Libre de Bruxelles, CP 620, 808, Route de Lennik, 1070, Brussels, Belgium
    Endocrine 32:197-205. 2007
    ..diabetes (CFRD), knowledge on the possible expression and cell distribution of the cystic fibrosis transmembrane conductance regulator (CFTR) protein within the endocrine pancreas is required...
  99. ncbi Role of NHERF1, cystic fibrosis transmembrane conductance regulator, and cAMP in the regulation of aquaporin 9
    Christine Pietrement
    Center for Systems Biology, Program in Membrane Biology Nephrology Division, Simches Research Center, Massachusetts General Hospital, 185 Cambridge Street, Boston, MA 02114, USA
    J Biol Chem 283:2986-96. 2008
    ..Here we show that NHERF1, cystic fibrosis transmembrane conductance regulator (CFTR), and AQP9 co-localize in the apical membrane of principal cells of the epididymis ..
  100. doi Involvement of apical P2Y2 receptor-regulated CFTR activity in muscarinic stimulation of Cl(-) reabsorption in rat submandibular gland
    Kazunari Ishibashi
    Department of Functional Bioscience, Fukuoka Dental College, Fukuoka, Japan
    Am J Physiol Regul Integr Comp Physiol 294:R1729-36. 2008
    ....
  101. doi Expression of cystic fibrosis transmembrane conductance regulator in ganglion cells of the hearts
    Peng Pan
    Department of Pathology, School of Basic Medical Sciences, Beijing University Health Science Center, Beijing, China
    Neurosci Lett 441:35-8. 2008
    b>Cystic fibrosis transmembrane conductance regulator (CFTR) as an important chloride-selective channel is known to distribute on the apical membrane of chloride-secreting epithelial cells...

Research Grants1

  1. Genetic epidemiology of glutathione and CF lung disease
    EDWARD MCKONE; Fiscal Year: 2005
    ..abstract_text> ..