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Sample search results for: a-dependent cftr chloride

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1 PHYSIOLOGICAL REVIEWS Vol. 79, Suppl., No. 1, January 1999
 

Summary:  coordinated behavior of the two NBDs is orchestrated by multiple protein kinase A-dependent phosphorylation... PHYSIOLOGICAL REVIEWS Vol. 79, Suppl., No. 1, January 1999 Printed in U.S.A. Control of CFTR... University, New York, New York I. Introduction S78 A. Domain organization of the CFTR molecule S78 B. ...

  

Source: Gadsby, David - Laboratory of Cardiac/Membrane Physiology, Rockefeller University

 

Collection: Biology and Medicine

 
2 The PDZ-interacting Domain of Cystic Fibrosis Transmembrane Conductance Regulator Is Required for Functional Expression in
 

Summary:  of CFTR in the apical membrane, and chloride secretion. Point substitution of the threonine (Thr... interaction between CFTR and EBP50, efficient expression of CFTR in the apical membrane as well as chloride... membrane, in- teraction of CFTR with EBP50, and for the ...

  

Source: Li, Min - Department of Neuroscience, Johns Hopkins University

 

Collection: Biology and Medicine

 
3 Abstract Cystic fibrosis (CF) is caused by mutations in the gene encoding the cystic fibrosis transmembrane
 

Summary:  - tions as an apical cAMP-regulated chloride channel in epithelial cells. The loss of CFTR activity in CF... treatment The CFTR protein is a cAMP-activated chloride channel that facilitates transepithelial chloride... or tobra- mycin treatment on cAMP-stimulated transepithelial ...

  

Source: Bedwell, David M. - Department of Microbiology, University of Alabama at Birmingham

 

Collection: Biology and Medicine

 
4 Introduction The cystic fibrosis transmembrane conductance regula-
 

Summary:  transmembrane conductance regulator (CFTR), a cAMP-activated chloride channel, to the apical plasma membrane... % of CFTR mutations, result in defective vectorial chloride transport, partly by altering the polarized... ­containing protein. We propose that COOH-terminal deletions of CFTR ...

  

Source: Li, Min - Department of Neuroscience, Johns Hopkins University

 

Collection: Biology and Medicine

 
5 Small Molecule Correctors of F508 CFTR Trafficking
 

Summary:  regulator gene (CFTR) that encodes a cAMP-regulated chloride ion channel expressed on the surface... Small Molecule Correctors of F508 CFTR Trafficking Therapeutic correctors of Cystic Fibrosis... Advantages: · Small molecule correctors of mutated chloride ion channels manage cystic fibrosis

  

Source: Michelson, David G. - Department of Electrical and Computer Engineering, University of British Columbia

 

Collection: Engineering

 
6 Genetic Disorders of Membrane Transport II. Regulation of CFTR by small molecules including HCO3
 

Summary:  derivatives as activators of the CFTR chloride channel. Br. J. Pharmacol. 123: 683­693, 1998. 5. Clarke, L. L... . Hwang. The CFTR chloride channel of mammalian heart. Annu. Rev. Physiol. 57: 387­416, 1995. 12. Gribkoff... . Regulation of chloride/bicarbonate exchange activity by ...

  

Source: Machen, Terry E. - Department of Molecular and Cell Biology, University of California at Berkeley

 

Collection: Biology and Medicine

 
7 PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X
 

Summary:  conductance regulator (CFTR) gene. The CFTR gene encodes a cAMP-activated chloride channel that is expressed... Activity in Cftr / hCFTR-G542X Mice. The hCFTR protein is a cAMP-activated chloride channel... Ex- pression and cAMP-Stimulated ...

  

Source: Bedwell, David M. - Department of Microbiology, University of Alabama at Birmingham

 

Collection: Biology and Medicine

 
8 Cystic fibrosis transmembrane conductance regulator in teleost fish W.S. Marshalla,*, T.D. Singerb
 

Summary:  distribution of CFTR in apical crypts of chloride cells. There was no detectable immunofluorescence deeper... , CFTR colocalizes with the chaperonin HSP60 (a 60-kDa heat shock protein) in chloride cells of gill... ]. In animals Fig. 4. Immunohistochemical staining of CFTR in ...

  

Source: Marshall, William S. - Department of Biology, St. Francis Xavier University

 

Collection: Environmental Sciences and Ecology

 
9 Interaction between CFTR and prestin (SLC26A5) Kazuaki Homma a
 

Summary:  online 6 February 2010 Keywords: CFTR Prestin SLC26A Chloride Outer hair cell Nonlinear capacitance... Cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-activated chloride channel... confirmed a prestin­CFTR interaction. Because chloride is ...

  

Source: Dallos, Peter - Department of Neurobiology and Physiology, Northwestern University

 

Collection: Biology and Medicine

 
10 Epithelial Sodium Channels Regulate Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channels in
 

Summary:  Epithelial Sodium Channels Regulate Cystic Fibrosis Transmembrane Conductance Regulator Chloride... , University of Iowa, Iowa City, Iowa 52242 The cystic fibrosis transmembrane conductance regu- lator (CFTR... ), in addition to its well defined Cl channel properties, regulates other ion channels. CFTR inhibits epithelial

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
11 Technical advance TheJournalofClinicalInvestigation http://www.jci.org Volume120 Number9 September2010 3149
 

Summary:  CFTR-knockout ferrets demonstrate defects in cAMP-induced chloride permeability and submucosal gland... September2010 3149 Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis Xingshen Sun,1... College of Medicine, University of Iowa, Iowa City, Iowa, USA. ...

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
12 Cell, Vol. 103, 169179, September 29, 2000, Copyright 2000 by Cell Press Accessory Protein Facilitated CFTR-CFTR
 

Summary:  Facilitated CFTR-CFTR Interaction, a Molecular Mechanism to Potentiate the Chloride Channel Activity 1989... ). The cloned CFTR gene encodes a chloride chan- nel that is activated by phosphorylation of the R domain by c... transmembrane conductance regu- lator (CFTR) gene encodes a ...

  

Source: Li, Min - Department of Neuroscience, Johns Hopkins University

 

Collection: Biology and Medicine

 
13 Interplay between ER Exit Code and Domain Conformation in CFTR Misprocessing and Rescue
 

Summary:  1 Interplay between ER Exit Code and Domain Conformation in CFTR Misprocessing and Rescue Gargi Roy... .wang@utoledo.edu Running title: Conformational Rescue of F508 CFTR Keywords: CFTR; conformation; F508; endoplasmic... reticulum; exit code #12;2 Multiple mutations in cystic fibrosis transmembrane conductance regulator ...

  

Source: Brand, Paul H. - Department of Physiology and Pharmacology, University of Toledo

 

Collection: Biology and Medicine

 
14 Pflgers Arch -Eur J Physiol (2001) 443 [Suppl1]: S62S64 DOI 10.1007/s004240100646
 

Summary:  , which are not required for functional expression of the CFTR chloride conductance, are also highly con... protein folding to the function of CFTR. CFTR is thought to form a chloride channel activated by c... , Bennett MK, Benos DJ, Quick MW, Kirk KL (1997) Regulation of ...

  

Source: Li, Min - Department of Neuroscience, Johns Hopkins University

 

Collection: Biology and Medicine

 
15 The Rockefeller University Press, 0021-9525/98/11/645/13 $2.00 The Journal of Cell Biology, Volume 143, Number 3, November 2, 1998 645657
 

Summary:  Abstract. The cystic fibrosis transmembrane conduc- tance regulator (CFTR) is a chloride channel that is de... a novel chloride sensor mecha- nism by which CFTR is capable of responding to changes in the extracellular... to a chloride sensor at which Cl binding facilitates structural ...

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
16 Abstract The activity of the CFTR Cl channel is de-pendent on its phosphorylation status set by kinases and
 

Summary:  dephosphorylation of different phosphoryla- tion sites by PP2B. Key words CFTR gating · Chloride transport... Abstract The activity of the CFTR Cl­ channel is de- pendent on its phosphorylation status set... ) are potential regulators of the cystic fibrosis conductance regulator (CFTR). Treating ...

  

Source: Machen, Terry E. - Department of Molecular and Cell Biology, University of California at Berkeley

 

Collection: Biology and Medicine

 
17 A Golgi-associated PDZ Domain Protein Modulates Cystic Fibrosis Transmembrane Regulator Plasma Membrane Expression*
 

Summary:  - cipitation experiments suggest that CAL exists as a mul- timer. Overexpression of CAL reduces CFTR chloride... cell surface expression of CFTR and chloride currents. In addition, NHE-RF inhibits the binding of CAL... conductance regulator (CFTR)1 (1­3). CFTR is a ...

  

Source: Li, Min - Department of Neuroscience, Johns Hopkins University

 

Collection: Biology and Medicine

 
18 ORIGINAL ARTICLE Suppression of CFTR premature termination codons
 

Summary:  with a cAMP-stimulated chloride current induced by the CFTR channel and is also inhibited by the CFTR... to increase CFTR channel open probability and enhance CFTR-dependent chloride secretion [25]. These cells also... clamp conditions. In the setting of a ...

  

Source: Bedwell, David M. - Department of Microbiology, University of Alabama at Birmingham

 

Collection: Biology and Medicine

 
19 J Mol Med (2006) 84: 573582 DOI 10.1007/s00109-006-0045-5
 

Summary:  chloride conductance could be detected in intestinal tissues of Cftr-/- hCFTR- G542X mice that had been... AMP-dependent transepithelial chloride currents. Figure 4 shows representative short-circuit current tracings obtained from Cftr... in the "Materials and methods" Table 1 ...

  

Source: Bedwell, David M. - Department of Microbiology, University of Alabama at Birmingham

 

Collection: Biology and Medicine

 
20 A divergent CFTR homologue: highly regulated salt transport in the euryhaline teleost F. heteroclitus
 

Summary:  and the chloride cell. In addition to the expected high level of kfCFTR expression in the gill and opercular... that CFTR is a chloride channel by alterations of its anion selectivity. Science 253: 202­205, 1991. 2... A divergent CFTR homologue: highly regulated salt transport in the ...

  

Source: Marshall, William S. - Department of Biology, St. Francis Xavier University; Tucker, Stephen J. - Department of Physics, University of Oxford

 

Collection: Biology and Medicine ; Environmental Sciences and Ecology ; Physics


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21 ORIGINAL ARTICLE A Mathematical Model of the Pancreatic Duct Cell
 

Summary:  (NaK pump) on a chloride-impermeable basolateral membrane, CFTR on the luminal membrane with 0.2 to 1... , CFTR primarily provides a mechanism for chloride efflux into the lumen to serve as a con- tinual source... only with the CFTR closed (minimal chloride conduc- ...

  

Source: Ermentrout, G. Bard - Center for the Neural Basis of Cognition & Department of Mathematics, University of Pittsburgh

 

Collection: Biology and Medicine ; Mathematics

 
22 HUMAN GENE THERAPY 16:11161123 (September 2005) Mary Ann Liebert, Inc.
 

Summary:  CFTR chloride conductance in polarized human CF air- way epithelial cells, using a SMaRT approach. AAV... ) to inhibit non-CFTR chloride channels, (3) 10 M foskolin/100 M 3-isobutyl-l-methylxanthine (IBMX) to activate... CFTR channels, and (4) 100 M bumetanide to inhibit all transep- ...

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
23 Expression of the Cystic Fibrosis Gene in Adult Human Lung John F. Engelhardt, * Monica Zepeda, * Jonathan A. Cohn, James R. Yankaskas, and James M. Wilson *
 

Summary:  regulator (CFTR), is a chloride channel that is pri- marily localized to the apical surface ofepithelial... - dence for the existence ofthe CFTR chloride channel has been demonstrated in surface epithelial cells... be functionally related in their capacity to facilitate CFTR-me- diated ...

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
24 # 2008 The Authors Journal compilation # 2008 Blackwell Munksgaard
 

Summary:  , DF508, a chloride channel [the cystic fibrosis transmem- brane conductance regulator (CFTR... 508, celastrol, CFTR, chemical biology, chloride conductance, cystic fibrosis, endoplasmic retic- ulum... to Temperature-Sensitive DF508 CFTR Trafficking Xiaodong Wang1,2, *, Atanas V. Koulov3 , ...

  

Source: Brand, Paul H. - Department of Physiology and Pharmacology, University of Toledo

 

Collection: Biology and Medicine

 
25 Introduction The cystic fibrosis transmembrane conductance regu-
 

Summary:  ­regulated) chloride conductance that is not mediated by CFTR. To deter- mine the specificity of the effects... are specif- ic for CFTR-mediated chloride currents. Discussion Previous studies have shown that syntaxin 1A... regu- lates CFTR chloride channels and ...

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
26 THEJOURNALOFCELLBIOLOGY The Rockefeller University Press $8.00
 

Summary:  The cystic fibrosis (CF) transmembrane conductance regulator (CFTR) is a cAMP-regulated chloride channel... common mutant form of the CF transmembrane conductance regulator (CFTR) F508 fails to exit... the endoplasmic reticulum (ER). Export of wild-type CFTR from the ER requires the coat complex II (COPII

  

Source: Brand, Paul H. - Department of Physiology and Pharmacology, University of Toledo

 

Collection: Biology and Medicine

 
27 Mitochondria-rich chloride (MR) cells of the skin and gill epithelia of teleost fish are responsible for ion uptake in fresh
 

Summary:  by monoclonal antibody directed to the C terminus of killifish CFTR (kfCFTR) in chloride cells of fresh water... Redistribution of immunofluorescence of CFTR anion channel and NKCC cotransporter in chloride cells during... in mitochondria-rich (MR) chloride cells in ...

  

Source: Marshall, William S. - Department of Biology, St. Francis Xavier University

 

Collection: Environmental Sciences and Ecology

 
28 Cystic Fibrosis Transmembrane Conductance Regulator-dependent Up-regulation of Kir1.1 (ROMK) Renal K Channels by the
 

Summary:  AMP- activated CFTR chloride current) that was not observed in water-injected control oocytes (data not shown... inhibition by the chloride channel blocker DPC (1 mM) confirms that IIBMX FSK is mediated by CFTR. In ENa... for Na absorption and K secretion. The cystic fibrosis trans- membrane conductance ...

  

Source: Tucker, Stephen J. - Department of Physics, University of Oxford

 

Collection: Physics ; Biology and Medicine

 
29 Evaluation of potential regulatory elements identied as DNase I hypersensitive sites in the CFTR gene
 

Summary:  of chloride secretion. The increased CFTR protein has been shown in part to be the result of transcriptional... Evaluation of potential regulatory elements identi®ed as DNase I hypersensitive sites in the CFTR... , Lyngby, Denmark The cystic ®brosis transmembrane conductance regulator (CFTR) gene shows a ...

  

Source: Ussery, David W. - Center for Biological Sequence Analysis, Institute of Biotechnology, Danmarks Tekniske Universitet

 

Collection: Biotechnology

 
30 Chloride Channels Myles H Akabas,Columbia University, New York, USA
 

Summary:  . A Chloride Channel from the Electric Organ of Torpedo . The ClC Family of Chloride Channels . CFTR... ­ a Regulated Chloride Channel . Regulation of CFTR by Phosphorylation and by Nucleotide Binding... (CFTR) (Figure 3b) and the GABA- and glycine-gated ...

  

Source: Akabas, Myles - Department of Physiology and Biophysics, Albert Einstein College of Medicine, Yeshiva University

 

Collection: Biology and Medicine

 
31 Mol. Biol. Evol. 18(9):17711788. 2001 2001 by the Society for Molecular Biology and Evolution. ISSN: 0737-4038
 

Summary:  . 1990; Gregory et al. 1991). More recently, the different roles of NBD1 and NBD2 in the CFTR chloride... identified in the cystic fibrosis transmembrane conductance regulator (CFTR) gene in classic and atypical... comparison of all currently available CFTR homologs, are likely to refine the global structure- ...

  

Source: Cutler, Chris - Department of Biology, Georgia Southern University

 

Collection: Biology and Medicine ; Environmental Sciences and Ecology

 
32 for publication Wiederhold,
 

Summary:  it is a channel (for chloride ions) rather than a transporter. Mutations in CFTR cause cystic fibrosis (Kerem et... CFTR Galileo: How old is this discovery? Ludovico: I don't think it was more than a few days old when I... characterization of CFTR (the cystic fibrosis trans- membrane conductance regulator) ...

  

Source: Groningen, Rijksuniversiteit - Centre for Ecological and Evolutionary Studies, Department of Marine Benthic Ecology and Evolution,

 

Collection: Biology and Medicine ; Environmental Sciences and Ecology

 
33 # 2007 The Authors Journal compilation # 2007 Blackwell Publishing Ltd
 

Summary:  conductance regulator (CFTR) is an apical membrane chloride channel implicated in cystic fibrosis... , it is unknown whether myosin VI plays a physiological role in regulating CFTR endocytosis in chloride... -0854.2007.00587.x Traffic 2007; 8: 998­1006 Blackwell Munksgaard Defective CFTR ...

  

Source: Apodaca, Gerard - Departments of Medicine & Cell Biology and Physiology, University of Pittsburgh

 

Collection: Biology and Medicine

 
34 RESEARCHHORIZONS esearchers have discov-
 

Summary:  with a crucial chloride chan- nel. This research was funded by the National Institutes of Health, National... inhibits a chlo- ride channel of known molecular identity." Chloride channels are crucial for secretion... do know that chloride channels open to allow millions of chloride ions to travel through ...

  

Source: Sherrill, David - School of Chemistry and Biochemistry, Georgia Institute of Technology

 

Collection: Chemistry

 
35 ENHANCEMENT OF ALVEOLAR EPITHELIAL SODIUM CHANNEL ACTIVITY WITH DECREASED CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR
 

Summary:  at Birmingham, Birmingham, AL 35205 Running title: CFTR decreases ENaC activity in alveolar cells in situ... the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) regulates the activity of amiloride sensitive... sodium channels (ENaC) in alveolar epithelial cells of wild type, heterozygous (Cftr (+/-)), knockout

  

Source: Bedwell, David M. - Department of Microbiology, University of Alabama at Birmingham

 

Collection: Biology and Medicine

 
36 BioMed Central Page 1 of 10
 

Summary:  study demonstrates for the first time that rat tracheal smooth cells express CFTR chloride channel... activation of CFTR chloride channels activity in rat airway smooth muscle cellsFigure 3 Pharmacological... activation of CFTR chloride channels activity in rat ...

  

Source: Ecole Polytechnique, Centre de mathématiques

 

Collection: Mathematics

 
37 Cystic Fibrosis Transmembrane Conductance Regulator
 

Summary:  CHLORIDE CHANNEL* Myles H. Akabas From the Center for Molecular Recognition, Departments of Physiology... transmembrane conductance regulator (CFTR)1 forms a Cl channel that is an essential component of epithelial Cl... in the basolateral mem- brane and exits through CFTR in the apical membrane; water follows osmotically (1

  

Source: Akabas, Myles - Department of Physiology and Biophysics, Albert Einstein College of Medicine, Yeshiva University

 

Collection: Biology and Medicine

 
38 Aminoglycosides are antibiotics that contain amino sugars linked to an ami-
 

Summary:  to function as chloride channels because of a regula- tory problem. Class 4 mutations also result in a CFTR... fibrosis transmembrane conductance regulator (CFTR) protein. CFTR is a member of the ATP-binding cassette... , frameshifts, in-frame deletions, and splicing mutations. The mutations all obviate ...

  

Source: Bedwell, David M. - Department of Microbiology, University of Alabama at Birmingham

 

Collection: Biology and Medicine

 
39 This Article Email this article to a colleague
 

Summary:  kinase A (PKA) still activates CFTR chloride channel after Mutagenesis of all 10 PKA consensus... of human CFTR (GenBank accession no. P13569) (1) and the Sav1866 exporter (3). Predicted membrane... -embedded regions of Sav1866 are colored green, and those of CFTR are blue. Coupling helices are red. Fig. 6

  

Source: Dokholyan, Nikolay V. - Department of Biochemistry and Biophysics, University of North Carolina at Chapel Hill

 

Collection: Physics ; Biology and Medicine

 
40 Spatiotemporal Coupling of cAMP Transporter to CFTR Chloride Channel
 

Summary:  Spatiotemporal Coupling of cAMP Transporter to CFTR Chloride Channel Function in the Gut Epithelia... fibrosis transmembrane conductance regulator (CFTR) is a cAMP-regulated chloride channel localized... , functionally and physically associates with CFTR. Adenosine-stimulated ...

  

Source: Li, Min - Department of Neuroscience, Johns Hopkins University

 

Collection: Biology and Medicine


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41 Poly-L-aspartic Acid Enhances and Prolongs Gentamicin-mediated Suppression of the CFTR-G542X
 

Summary:  AMP-activated chloride channel, and transepithelial chloride conductance attributable to CFTR can be monitored... the magnitude of cAMP-stimulated transepi- thelial chloride currents resulting from the endogenous mouse CFTR... Poly-L-aspartic Acid Enhances and Prolongs Gentamicin-mediated ...

  

Source: Bedwell, David M. - Department of Microbiology, University of Alabama at Birmingham

 

Collection: Biology and Medicine

 
42 Multiple Membrane-Cytoplasmic Domain Contacts in the Cystic Fibrosis Transmembrane Conductance Regulator
 

Summary:  Regulator (CFTR) Mediate Regulation of Channel Gating*S Received for publication,May 21, 2008... fibrosis transmembrane conductance regulator (CFTR) is a unique ATP-binding cassette (ABC) ion channel... of the CFTR three-dimensional structure, supported by experimental data (Serohijos, A. W., Hegedus, T

  

Source: Dokholyan, Nikolay V. - Department of Biochemistry and Biophysics, University of North Carolina at Chapel Hill

 

Collection: Physics ; Biology and Medicine

 
43 Bioelectric Properties of Chloride Channels in Human, Pig, Ferret, and Mouse Airway Epithelia
 

Summary:  demonstrated cAMP- inducible, non­CF transmembrane conductance regulator (non- CFTR) chloride transport... ) in the airway (7, 8), and/or the alternative expression or activation of pathways that control non-CFTR chloride... functional differences in the activities of ENaC, CFTR, and/or ...

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
44 Technical advance TheJournalofClinicalInvestigation http://www.jci.org Volume 118 Number 4 April 2008 1571
 

Summary:  .E., Stutts, J., and Guggino, W.B. 1999. CFTR is a conductance regulator as well as a chloride channel... .L., and Galietta, L.J. 2006. CFTR chloride channel drug discovery -- inhibitors as antidiarrheals and activators... April 2008 1571 Production of CFTR-null and ...

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
45 49 J. Gen. Physiol. The Rockefeller University Press 0022-1295/99/07/49/5 $5.00 Volume 114 July 1999 4953
 

Summary:  s Anderson, M.P., and M.J. Welsh. 1992. Regulation by ATP and ADP of CFTR chloride channels that contain... .J. Gregory, A.E. Smith, and M.J. Welsh. 1991. Nucleoside triphosphates are required to open the CFTR chloride... :28463­28468. Mathews, C.J., J.A. Tabcharani, and J.W. Hanrahan. 1998. The ...

  

Source: Gadsby, David - Laboratory of Cardiac/Membrane Physiology, Rockefeller University

 

Collection: Biology and Medicine

 
46 Dual Therapeutic Utility of Proteasome Modulating Agents for Pharmaco-Gene Therapy of the
 

Summary:  population. CFTR is a chloride channel that localizes to the apical membrane of epithelial cells in many... - dependent phosphorylation. In addition to functioning as a chloride channel, CFTR has also been shown... in complementing CFTR chloride transport defects in ...

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
47 1992 Oxford University Press Human Molecular Genetics, Vol. 1, No. 2 77--82 Identification and developmental expression of the
 

Summary:  into lipid bilayers (14), provides strong evidence that the CFTR gene product possesses intrinsic chloride... . Three of the four PKA sites implicated in the regulation of the human CFTR chloride channel in vivo... . If CFTR functions as a chloride channel in these ...

  

Source: Tucker, Stephen J. - Department of Physics, University of Oxford

 

Collection: Physics ; Biology and Medicine

 
48 Conductance Regulator Bicarbonate Conductance and pH Regulatory Capability of Cystic Fibrosis Transmembrane
 

Summary:  - transfected cells also exhibit a low conductance, cAMP- and protein kinase A-dependent Cl- channel that has... transmembrane conduc- tance regulator (CFTR) is an epithelial Cl- channel regulated by protein kinase A... . The most common mutation In cystic fibrosis (CF), deletion ofPhe-S08 (AF508-CFTR), reduces Cl1 secretion

  

Source: Machen, Terry E. - Department of Molecular and Cell Biology, University of California at Berkeley

 

Collection: Biology and Medicine

 
49 UNIVERSITE Joseph Fourier, Grenoble 1 cole Doctorale Chimie et Sciences du Vivant
 

Summary:  la protéine CFTR Soutenue publiquement le 2 Juillet 2008 Composition du jury : Prof. Frédéric BECQ... cftr................................................8 A. LA MUCOVISCIDOSE... ...........................................................14 III. LA PROTÉINE ...

  

Source: Ecole Polytechnique, Centre de mathématiques

 

Collection: Mathematics

 
50 Technical advance 1578 TheJournalofClinicalInvestigation http://www.jci.org Volume 118 Number 4 April 2008
 

Summary:  of the CFTR chloride channel in ferret airway epithelia are also similar to those seen in human airway... 4 April 2008 Adeno-associated virus­targeted disruption of the CFTR gene in cloned ferrets... -associatedvirus­mediatedgenetargetinginfibroblasts,followedbynucleartransfercloning. ...

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
51 H U M A N G E N E T H E R A P Y 5:615-639 (1994) Mary Ann Liebert, Inc., Publishers
 

Summary:  . Abstract Cystic fibrosis (CF) is an autosomal recessive disease that reflects mutations in the CFTR gene... . Multiple mutations in this gene have been detected that lead to a protein (CFTR) that is abnormally... defined, but it is clear that CFTR can act as a cAMP-regulated Cl~ channel. This type of defect

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
52 Hsp90 Cochaperone Aha1 Downregulation Rescues Misfolding
 

Summary:  (CFTR) is a multidomain cAMP-regulated chloride channel found in the apical mem- brane of polarized... Hsp90 Cochaperone Aha1 Downregulation Rescues Misfolding of CFTR in Cystic Fibrosis Xiaodong Wang,1... ) transmembrane con- ductance regulator (CFTR) protein interactions (the CFTR ...

  

Source: Brand, Paul H. - Department of Physiology and Pharmacology, University of Toledo

 

Collection: Biology and Medicine

 
53 Annu. Rev. Physiol. 2005. 67:71958 doi: 10.1146/annurev.physiol.67.032003.154341
 

Summary:  , CFTR Abstract Calcium-activated chloride channels (CaCCs) play important roles in cellular physiology... -ACTIVATED CHLORIDE CHANNELS 733 example, values for CFTR range from 0.003 to 0.2 (see 181). Other anion channels... CALCIUM-ACTIVATED CHLORIDE CHANNELS Criss Hartzell,1 Ilva ...

  

Source: Hartzell, Criss- Department of Cell Biology, Emory University

 

Collection: Biology and Medicine

 
54 Phenylalanine-508 mediates a cytoplasmicmembrane domain contact in the CFTR 3D structure crucial
 

Summary:  ) Assembly of functional CFTR chloride channels. Annu Rev Physiol 67:701­718. 7. Sharma M, Benharouga M, Hu W... Phenylalanine-508 mediates a cytoplasmic­membrane domain contact in the CFTR 3D structure crucial... (NBD1) of the cystic fibrosis transmem- brane conductance regulator (CFTR), a member of the ATP

  

Source: Dokholyan, Nikolay V. - Department of Biochemistry and Biophysics, University of North Carolina at Chapel Hill

 

Collection: Physics ; Biology and Medicine

 
55 Alternative Splicing at a NAGNAG Acceptor Site as a Novel Phenotype Modifier
 

Summary:  patients homozygous for the E831X mutation (2623G.T) in the CFTR gene. Analyses performed on nasal... fibrosis transmembrane conductance regulator gene (CFTR). Querying the Tandem Splice Site DataBase (Tass... in CFTR. Mutations in the CFTR gene, which encodes a cAMP-regulated Cl2 channel located at the ...

  

Source: Ecole Polytechnique, Centre de mathématiques

 

Collection: Mathematics

 
56 TheJournalofGeneralPhysiology J. Gen. Physiol. The Rockefeller University Press 0022-1295
 

Summary:  for activation of CFTR chloride current by PKA is shifted to higher [PKA] for WT channels than for mutant S768A... -domain Serine 768 Dampens Activation of CFTR Channels by PKA László Csanády,1,2 Donna Seto-Young,2 Kim W. Chan,2... , New York, NY 10021 abstract CFTR (cystic fibrosis transmembrane ...

  

Source: Chait, Brian T. - Laboratory of Mass Spectrometry and Gaseous Ion Chemistry, Rockefeller University

 

Collection: Biology and Medicine

 
57 TheJournalofGeneralPhysiology 333 J. Gen. Physiol. The Rockefeller University Press 0022-1295/2003/09/333/16 $8.00
 

Summary:  Nonhydrolytic Interaction of Nucleotide with CFTR's NH2-terminal Nucleotide Binding Domain and its Role... University, New York, NY 10021 abstract CFTR, the protein defective in cystic fibrosis, functions as a Cl... channel regulated by cAMP-depen- dent protein kinase (PKA). CFTR is also an ATPase, comprising two

  

Source: Gadsby, David - Laboratory of Cardiac/Membrane Physiology, Rockefeller University

 

Collection: Biology and Medicine

 
58 Peptide binding consensus of the NHE-RF-PDZ1 domain matches the C-terminal sequence of cystic brosis transmembrane conductance
 

Summary:  in addition to Na -H exchangers. Besides activation by protein kinase A, the CFTR chloride channel activity... ¢brosis transmembrane conductance regulator (CFTR) Shusheng WangY , Ronald W. Raab , Peter J. Schatz... and found that NHE-RF-PDZ1 is capable of binding to the CFTR C-terminus. The specific and ...

  

Source: Li, Min - Department of Neuroscience, Johns Hopkins University

 

Collection: Biology and Medicine

 
59 Comprehensive and Rapid Genotyping of Mutations and Haplotypes in Congenital Bilateral Absence of the Vas Deferens and Other Cystic Fibrosis Transmembrane
 

Summary:  fibrosis transmembrane conductance regulator (CFTR) mutations causing classic cystic fibrosis... and for the Tn variant in IVS8. However, full scanning of CFTR is needed for the diagnosis of patients... with cystic fibrosis or CFTR-related disorders (including congenital bilateral absence of the vas deferens

  

Source: Ecole Polytechnique, Centre de mathématiques

 

Collection: Mathematics

 
60 163 J. Gen. Physiol. The Rockefeller University Press 0022-1295/2000/08/163/18 $5.00 Volume 116 August 2000 163180
 

Summary:  regulator is a Cl channel that belongs to the family of ATP-binding cassette proteins. The CFTR polypeptide... and by ATP binding, and, likely, hydrolysis at the NBDs. Exon 13 of the CFTR gene encodes amino acids (aa... 's) 590­830, which were originally ascribed to the R domain. In this study, CFTR channels were severed

  

Source: Gadsby, David - Laboratory of Cardiac/Membrane Physiology, Rockefeller University

 

Collection: Biology and Medicine


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61 Coupled secretion of chloride and mucus in skin of Xenopus Zaevis: possible role for CFTR
 

Summary:  Coupled secretion of chloride and mucus in skin of Xenopus Zaevis: possible role for CFTR JOHN F... Physiological Society c491 #12;c492 CFTR-MEDIATED COUPLED CHLORIDE AND MUCUS SECRETION 1,795 bp, 3'-primer 2... was abolished #12;c494 CFTR-MEDIATED COUPLED CHLORIDE ...

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
62 TheJournalofGeneralPhysiology 17 J. Gen. Physiol. The Rockefeller University Press 0022-1295/2003/01/17/20 $8.00
 

Summary:  of MgATP-dependent Gating of CFTR Cl Channels Paola Vergani,1 Angus C. Nairn,2 and David C. Gadsby1 1... University, New York, NY 10021 abstract CFTR, the product of the gene mutated in cystic fibrosis... molecular mechanisms remain controversial. To investigate them, we expressed CFTR channels in Xenopus

  

Source: Gadsby, David - Laboratory of Cardiac/Membrane Physiology, Rockefeller University

 

Collection: Biology and Medicine

 
63 Channel-Lining Residues in the M3 Membrane-Spanning Segment of the Cystic Fibrosis Transmembrane Conductance Regulator
 

Summary:  ABSTRACT: The cystic fibrosis transmembrane conductance regulator (CFTR) forms a chloride-selective channel... that is involved in chloride transport in a variety of epithelia and other cells (2, 3). CFTR also appears... of CFTR have been exten- sively studied. CFTR ...

  

Source: Akabas, Myles - Department of Physiology and Biophysics, Albert Einstein College of Medicine, Yeshiva University

 

Collection: Biology and Medicine

 
64 291:218-230, 2006. doi:10.1152/ajpcell.00605.2005Am J Physiol Cell Physiol Terry E. Machen
 

Summary:  ] , February 1, 2007; 292 (2): L381-L382.Am J Physiol Lung Cell Mol Physiol B. K. Rubin CFTR is a modulator... conductance regulator (CFTR) in the apical membranes of CF airway epithelial cells abolishes c... and then review proposals that might explain how reduced CFTR function could activate such proinflammatory

  

Source: Machen, Terry E. - Department of Molecular and Cell Biology, University of California at Berkeley

 

Collection: Biology and Medicine

 
65 HUMAN GENE THERAPY 9:635-648 (March 20, 1998) Mary Ann Liebert, Inc.
 

Summary:  conductance regulator (CFTR) gene, which encodes an epithelial chloride (CI") channel (Kerem et al, 1989... (CFTR) gene. These defects include al- tered Cl~ and Na"*" permeability as well as intracellular defects... with a single dysfunctional gene. OVERVIEW SUMMARY some/DNA complex-mediated CFTR gene ...

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
66 545 J. Gen. Physiol. The Rockefeller University Press 0022-1295/2002/06/545/15 $5.00 Volume 119 June 2002 545559
 

Summary:  Limit Opening and Closing of a Single CFTR Cl Channel Athanasios G. Dousmanis,1 Angus C. Nairn,2... regulator (CFTR) Cl channels remain controversial. In this work, the contributions made by ATP and Mg2 ions... to the gating of phosphorylated cardiac CFTR channels were evaluated separately by measuring the rates

  

Source: Gadsby, David - Laboratory of Cardiac/Membrane Physiology, Rockefeller University

 

Collection: Biology and Medicine

 
67 doi:10.1152/ajpcell.00127.2009 297:750-758, 2009. First published Jul 1, 2009;Am J Physiol Cell Physiol
 

Summary:  of chloride channel blockers on the cardiac CFTR chloride and L-type calcium currents. Cardiovasc Res 32: 391... fibrosis transmembrane regulator (CFTR) Cl conductance blocked depolarization of GI neurons to decreased... glucose or AMPK activation, whereas decreased glucose, AMPK activation, and 8-Br- cGMP ...

  

Source: Routh, Vanessa H. - Department of Pharmacology and Physiology, University of Medicine and Dentistry of New Jersey

 

Collection: Biology and Medicine

 
68 Arg352 Is a Major Determinant of Charge Selectivity in the Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel
 

Summary:  Conductance Regulator Chloride Channel Romain Guinamard and Myles H. Akabas*,,§ Center for Molecular... conductance regulator (CFTR)1 is a member of the ATP-binding-cassette membrane transporter gene superfamily... . It forms a small-conductance, chloride channel that is gated by protein kinase A-mediated phosphorylation

  

Source: Akabas, Myles - Department of Physiology and Biophysics, Albert Einstein College of Medicine, Yeshiva University

 

Collection: Biology and Medicine

 
69 Rapid Publication A Mouse Model for the AF508 Allele of Cystic Fibrosis
 

Summary:  is a mutation that deletes phenylalanine 508 in cystic fibrosis transmembrane conductance regulator (CFTR... a novel strategy to introduce the AF508 mutation into the mouse CFTR gene. Affected epi- thelia from... homozygous AF508 mice lacked CFTR in the apical membrane and were Cl- impermeable. These abnor- malities

  

Source: Capecchi, Mario R. - Department of Biology, University of Utah

 

Collection: Biology and Medicine

 
70 Vol. 333 No. 13 ADENOVIRAL-VECTORMEDIATED GENE TRANSFER FOR CYSTIC FIBROSIS 823 A CONTROLLED STUDY OF ADENOVIRAL-VECTORMEDIATED GENE TRANSFER IN THE
 

Summary:  . Each lot was efficacious for CFTR transduction by the Western blot assay and for chloride secretion... response to perfusion with a chloride-free solution containing amiloride); cAMP-regulated (CFTR) secretion... the defect in chloride secretion.35,40 The PD protocols that are sensitive ...

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
71 289 J. Gen. Physiol. The Rockefeller University Press 0022-1295/97/03/289/11 $2.00 Volume 109 March 1997 289299
 

Summary:  Conductance Regulator (CFTR) Chloride Channel Min Cheung* and Myles H. Akabas*§ From the *Center for Molecular... t i o n Cystic fibrosis transmembrane conductance regulator (CFTR)1 forms a chloride channel whose... of CFTR due to the endogenous levels of cAMP. The ...

  

Source: Akabas, Myles - Department of Physiology and Biophysics, Albert Einstein College of Medicine, Yeshiva University

 

Collection: Biology and Medicine

 
72 281:633-648, 2001.Am J Physiol Cell Physiol and Sheldon S. Miller
 

Summary:  , Jonathan Cohn, Gary L. Firestone, Terry E. Machen in mammary epithelia ENaC- and CFTR-dependent ion... epithelia P2 purinoceptors regulate calcium-activated chloride and fluid transport in 31EG4 [PDF][Full Text... .physiology.orgDownloadedfrom #12;ENaC- and CFTR-dependent ion and fluid transport in mammary epithelia SASHA ...

  

Source: Machen, Terry E. - Department of Molecular and Cell Biology, University of California at Berkeley

 

Collection: Biology and Medicine

 
73 THEJOURNAL OF BIOLOGICALCHEMISTRY 0 1992 by TheAmerican Societyfor Biochemistryand MolecularBiology, Inc.
 

Summary:  that CFTR mRNA is expressed inlympho- cytes, consistentwith thecAMP regulation of chloride transport present... Chloride Current* (Receivedfor publication, August 14, 1991 Thomas V.McDonald$Bfl,Paul T. Nghiem... conductance regulator (CFTR), are responsible forthis disease. Whether a physiological defect ...

  

Source: Nghiem, Paul - Cutaneous Biology Research Center, Massachusetts General Hospital

 

Collection: Biology and Medicine

 
74 For personal use. Only reproduce with permission from The Lancet Publishing Group. 2014 THE LANCET Vol 358 December 15, 2001
 

Summary:  mutations result in CFTR proteins that do not function as chloride channels (such as the mutant G551D... pharmacologic restoration of CFTR-mediated chloride transport with sodium 4­phenylbutyrate in cystic fibrosis... conductance regulator (CFTR) protein, which is a member of the ...

  

Source: Bedwell, David M. - Department of Microbiology, University of Alabama at Birmingham

 

Collection: Biology and Medicine

 
75 Proc. Natl. Acad. Sci. USA Vol. 95, pp. 84968501, July 1998
 

Summary:  interest. Mutations in CFTR cause cystic fibrosis (18). CFTR is a chloride channel, but some cellular... , such as the purinergic P2Y1 receptor and cystic fibrosis transmem- brane conductance regulator (CFTR). MATERIALS... , Na H exchanger regulatory factor; CFTR, cystic fibrosis ...

  

Source: Hall, Randy A - Department of Pharmacology, Emory University

 

Collection: Biology and Medicine

 
76 Computational Studies Reveal Phosphorylation-dependent Changes in the
 

Summary:  regulator (CFTR) is a cAMP-dependent chloride channel that is mutated in cystic fibrosis, an inherited... - membrane conductance regulator (CFTR), a cAMP- regulated chloride channel that belongs to the ATP- binding... the chloride channel. This was demonstrated experimentally using a ...

  

Source: Dokholyan, Nikolay V. - Department of Biochemistry and Biophysics, University of North Carolina at Chapel Hill

 

Collection: Physics ; Biology and Medicine

 
77 Dr. Campbell's Bio111 In-Class Exam #2.5 Spring 2008 Spring 2008 Biology 111 Exam #2.5 KEY -Molecular Genetics In-Class Exam
 

Summary:  -type. In each picture, include mucus, chloride ions, CFTR. Label all parts and which cell is which. Looking... for mucus on one side only, Cl- ions in the wt mucus and not in the CF mucus, CFTR in the PM of wt cells

  

Source: Campbell, A. Malcolm - Biology Department, Davidson College

 

Collection: Multidisciplinary Databases and Resources ; Biology and Medicine

 
78 Proton leak and CFTR in regulation of Golgi pH in respiratory epithelial cells
 

Summary:  Proton leak and CFTR in regulation of Golgi pH in respiratory epithelial cells GRISCHA CHANDY,1... , and Terry E. Machen. Proton leak and CFTR in regulation of Golgi pH in respiratory epithelial cells. Am J... conductance regulator (CFTR) plays a role in regulating organelle pH has remained incon- clusive. We

  

Source: Grabe, Michael - Department of Biological Sciences, University of Pittsburgh; Machen, Terry E. - Department of Molecular and Cell Biology, University of California at Berkeley

 

Collection: Biology and Medicine

 
79 JOP. J. Pancreas (Online) 2001; 2(4 Suppl):229-236. JOP. Journal of the Pancreas http://www.joplink.net Vol.2, No.4 Suppl. July 2001 229
 

Summary:  conductance regulator- (CFTR)-corrected airway epithelial cells [1]. Digital imaging microscopy of these Golgi... -localized probes showed that there was no correlation between pHG (6.4-7.0) and the presence of CFTR, whether cells... were in HCO3 - /CO2-containing or in HCO3 - /CO2-free solutions. Activation of CFTR by raising ...

  

Source: Grabe, Michael - Department of Biological Sciences, University of Pittsburgh; Machen, Terry E. - Department of Molecular and Cell Biology, University of California at Berkeley

 

Collection: Biology and Medicine

 
80 Congenital bilateral absence of the vas
 

Summary:  LETTER Congenital bilateral absence of the vas deferens and recombination at CFTR European Journal... report a paucity of variation on V470 haplotypes at the CFTR locus relative to M470 haplotypes... proportion of transcripts containing exon 9 and the latter in a translated gene product with reduced chloride

  

Source: Haig, David - Department of Organismic and Evolutionary Biology, Harvard University

 

Collection: Environmental Sciences and Ecology


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81 RECENT RESEARCH CONTRIBUTIONS (Directly supervised Honours & Masters Students indicated with *)
 

Summary:  of immunofluorescence of CFTR anion channel and NKCC cotransporter in chloride cells during adaptation of the killifish... and SPAK in osmosensing chloride secreting cells of killifish opercular epithelium. J. exp. Biol. 2005... decrease response of the cell, through a cotransporter in the cell membrane sodium, potassium, ...

  

Source: Marshall, William S. - Department of Biology, St. Francis Xavier University

 

Collection: Environmental Sciences and Ecology

 
82 CFTR Displays Voltage Dependence and Two Gating Modes during Stimulation
 

Summary:  CFTR Displays Voltage Dependence and Two Gating Modes during Stimulation HORST FISCHER and TERRY E... fibroblast cells expressing the CFTR were stimulated in cell-attached mode with forskolin. The number (N... . Thus, during stimulation the CFTR increased its activity through two distinct effects: the reduction

  

Source: Machen, Terry E. - Department of Molecular and Cell Biology, University of California at Berkeley

 

Collection: Biology and Medicine

 
83 The Cdc42 inhibitor secramine B prevents cAMP-induced K+ conductance in intestinal epithelial cells
 

Summary:  Abbreviations: CaCC, calcium activated chloride channel cAMP, 30 ,50 -cyclic AMP CFTR, cystic fibrosis... (CFTR) or the Ca2+ -activated chloride channel (CaCC). Export of K+ across the basolateral membrane... translocation and Cdc42 activation via protein kinase A-dependent pathway. J ...

  

Source: Kirchhausen, Tomas - Immune Disease Institute & Department of Cell Biology, Harvard University

 

Collection: Biology and Medicine

 
84 A Journal of Integrative Biology Short-Term Low-Salinity Tolerance by the Longhorn
 

Summary:  À cotransporter (NKCC1), and the chloride channel, cystic fibrosis transmembrane conductance regulator (CFTR... ), 20% SW, or 10% SW for 24 or 72 hr. Plasma osmolality, sodium, potassium, and chloride concentrations... , there was not a downregulation of gill NKA, NKCC1, and CFTR mRNA or protein during ...

  

Source: Evans, David H. - Department of Biology, University of Florida

 

Collection: Environmental Sciences and Ecology

 
85 The CFTR Met 470 Allele Is Associated with Lower Birth Rates in Fertile Men from a Population Isolate
 

Summary:  conductance regulator (CFTR; OMIM 602421) gene functions as a chloride channel that regulates salt and water... The CFTR Met 470 Allele Is Associated with Lower Birth Rates in Fertile Men from a Population... regulator (CFTR) gene in reproductive physiology, numerous variants in this gene have been implicated

  

Source: Abney, Mark - Department of Human Genetics, University of Chicago; Das, Soma - Department of Human Genetics, University of Chicago

 

Collection: Biology and Medicine ; Computer Technologies and Information Sciences

 
86 Current Pharmacogenomics, 2005, 3, 000-000 1 1570-1603/05 $50.00+.00 2005 Bentham Science Publishers Ltd.
 

Summary:  (CFTR) protein. The CFTR protein is a cAMP-activated chloride channel [Riordan et al., 1989]. Loss... of CFTR activity results in abnormal chloride and sodium transport across the apical membrane... - glycoside concentration in a dose-dependent manner. In addition, cAMP-activated ...

  

Source: Bedwell, David M. - Department of Microbiology, University of Alabama at Birmingham

 

Collection: Biology and Medicine

 
87 Am. J. Respir. Cell Mol. Biol. Vol. 24, pp. 195202, 2001 Internet address: www.atsjournals.org
 

Summary:  implicate CFTR in the func- tional pathway leading to high levels of baseline, unstimu- lated chloride... channels, including Ca -acti- vated Cl channels, CFTR, and/or the outward rectifying chloride channel (ORCC... . Second, submucosal glands may secrete chloride-rich fluid into the ...

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
88 Species-Specific Differences in Mouse and Human Airway Epithelial Biology of Recombinant
 

Summary:  . Interest- ingly, cAMP-inducible chloride currents were enhanced in F508CFTR mouse ALI cultures, making... ) to activate CFTR chloride channels. Finally, bumetanide (100 M) was added to the basolateral chamber to block... airway epithelia reproduce defects in CFTR-mediated ...

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
89 MECHANISMS OF DISEASE Volume 336 Number 22 1575
 

Summary:  NO. OF AMINO ACIDS COMMON MUTATIONS Cystic fibrosis AR CFTR (epithelial chloride channel) 7q 1480 F... ) Activate non-CFTR chloride channels with aerosolized UTP (phase 3) Extracellular Intracellular Cell... of cystic fibrosis stem from a defect in a chlo- ride-channel protein, CFTR, that ...

  

Source: Clapham, David E. - Department of Neurobiology, Harvard University

 

Collection: Biology and Medicine

 
90 BioMed Central Page 1 of 7
 

Summary:  for the CF transmembrane conductance regulator (CFTR) protein, which acts as a chloride channel after... , and sweat chloride concentrations close to normal values. In vivo demonstration of abnormal CFTR protein... inhibitor amiloride, and CFTR-mediated chloride (Cl-) ...

  

Source: Ecole Polytechnique, Centre de mathématiques

 

Collection: Mathematics

 
91 477 J. Gen. Physiol. The Rockefeller University Press 0022-1295/2000/09/477/24 $5.00 Volume 116 September 2000 477500
 

Summary:  , The Rockefeller University, New York, New York 10021-6399 abstract Opening and closing of a CFTR Cl channel... and the two nucleotide binding domains were probed by character- izing the gating of severed CFTR channels... . The kinetic behavior of wild-type (WT) CFTR channels was compared with that of split ...

  

Source: Gadsby, David - Laboratory of Cardiac/Membrane Physiology, Rockefeller University

 

Collection: Biology and Medicine

 
92 Select Journal or Resource |Institution: UAB LISTER HILL LIB/UNIV HOSP Sign In as Individual
 

Summary:  regulator ( ) gene.CFTR Pilot studies involving patients with CF caused by this nonsense mutation hint... measure of protein function, a test that detects transport of chloride ions across the nasal epithelium... , a sign of restored gene expression ( 2000;161:860-865). CFTR CFTR CFTR Am ...

  

Source: Bedwell, David M. - Department of Microbiology, University of Alabama at Birmingham

 

Collection: Biology and Medicine

 
93 Seawater teleosts drink sea water and absorb ions and fluid across the oesophagus and intestine and in this way obtain
 

Summary:  not CFTR), had no effect on Gt, Isc or the net Cl­ flux. In the chloride cells of the opercular epithelium... in unidirectional fluxes and Gt. Cystic fibrosis transmembrane conductance regulator (CFTR) was immunocytochemically... appears to involve activation of CFTR ion channels in the apical membrane of a ...

  

Source: Marshall, William S. - Department of Biology, St. Francis Xavier University

 

Collection: Environmental Sciences and Ecology

 
94 Directed evolution of adeno-associated virus to an infectious respiratory virus
 

Summary:  chloride transport defect. CF airway epithelia were transduced with AAV2.5T encoding a shortened CFTR... R restored CFTR chloride current to normal levels (Fig. 4E, IsccAMP 12 4 A.cm 2 , IscGlyH 18 9 A... AMP-regulated chloride transport that is sensitive to the CFTR ...

  

Source: Long, Jeffrey R.- Department of Chemistry, University of California at Berkeley

 

Collection: Materials Science ; Chemistry

 
95 H U M A N G E N E T H E R A P Y 5:501-519 (1994) Mary Ann Liebert, Inc., Publishers
 

Summary:  Fibrosis Trans- membrane Conductance Regulator (CFTR) which appears to be a highly regulated chloride... delivering CFTR-expressing, replication- defective adenoviruses to the airway. The rationale for this human... xe- nografts and nonhuman primates. In this protocol, 20 CF patients will be treated with ...

  

Source: Engelhardt, John F. - Department of Anatomy and Cell Biology, University of Iowa

 

Collection: Biology and Medicine

 
96 Rapid regulation of NaCl secretion by estuarine teleost fish: coping strategies for short-duration freshwater exposures
 

Summary:  AMP-activated current [22]. In excised patches from chloride cells, PKA and ATP addition activate CFTR-like channels... , a demonstration of syntaxin 1A localization to chloride cells and binding to kfCFTR would be a first step... on the basolateral side and protein kinase A regulation of the ...

  

Source: Marshall, William S. - Department of Biology, St. Francis Xavier University

 

Collection: Environmental Sciences and Ecology

 
97 38 Dartmouth Medicine Fall 2000 Physiologist Bruce
 

Summary:  and chloride) across cell membranes. In lung cells, salt moves through chloride channels in the cell membrane... to the airway surface. Then, just as a dry sponge soaks up water, the chloride and sodium combination pulls... . People with one abnor- mal CF gene seem to have half as many secretory chloride ...

  

Source: Lotko, William - Thayer School of Engineering, Dartmouth College

 

Collection: Geosciences ; Engineering

 
98 Freshwater teleosts have extracellular fluids that are hyperosmotic to their environment, and they constantly gain
 

Summary:  pavement cell epithelium lacking chloride cells. Actin and CFTR The higher conductance per crypt... ). The apical location of actin in chloride cells is intriguing because this is also the apparent location of kfCFTR... binding to CFTR (for a review, see Kleizen et al., 2000); hence, the ...

  

Source: Marshall, William S. - Department of Biology, St. Francis Xavier University

 

Collection: Environmental Sciences and Ecology

 
99 UNIVERSIT JOSEPH FOURIER GRENOBLE I Thse prsente par
 

Summary:  et évaluation de modulateurs de la protéine CFTR Soutenue le 9 décembre 2008 Composition du jury Pr... moi sur la thématique des modulateurs de CFTR et avec qui j'ai partagé « la longueur des purif' des P1... ....................................................................... 18 3. La protéine CFTR (Sheppard, 1999 ; ...

  

Source: Ecole Polytechnique, Centre de mathématiques

 

Collection: Mathematics

 
100 Final Accepted Version G-00355-2005.R1 Chronic PKC activation in HT-29 Cl.19A colonocytes (1) Prevents cAMP-
 

Summary:  of the basolateral Na+ /K+ ATPase, Na+ /2Cl- /K+ co-transporter, and open apical membrane CFTR-chloride Page 3 of 51... stimulated a cAMP-dependent activation of CFTR-mediated chloride efflux (34) whereas, in colonocytes, PKCG... of CFTR mediated chloride secretion PKCD1 is the candidate ...

  

Source: Xiang, Kevin Y. - Department of Molecular and Integrative Physiology, University of Illinois at Urbana-Champaign

 

Collection: Biology and Medicine


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