Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells

Devra P. Rich; Matthew P. Anderson; Richard J. Gregory; Seng H. Cheng; Sucharita Paul; Douglas M. Jefferson; John D. McCann; Katherine W. Klinger; Alan E. Smith; Michael J. Welsh

doi:10.1038/347358a0

Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells

Nature ◽

10.1038/347358a0 ◽

1990 ◽

Vol 347 (6291) ◽

pp. 358-363 ◽

Cited By ~ 443

Author(s):

Devra P. Rich ◽

Matthew P. Anderson ◽

Richard J. Gregory ◽

Seng H. Cheng ◽

Sucharita Paul ◽

...

Keyword(s):

Cystic Fibrosis ◽

Epithelial Cells ◽

Chloride Channel ◽

Airway Epithelial Cells ◽

Transmembrane Conductance Regulator ◽

Airway Epithelial ◽

Transmembrane Conductance ◽

Channel Regulation ◽

Cystic Fibrosis Airway ◽

Cystic Fibrosis Transmembrane

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Spliceosome-Mediated RNATrans-Splicing with Recombinant Adeno-Associated Virus Partially Restores Cystic Fibrosis Transmembrane Conductance Regulator Function to Polarized Human Cystic Fibrosis Airway Epithelial Cells

Human Gene Therapy ◽

10.1089/hum.2005.16.1116 ◽

2005 ◽

Vol 16 (9) ◽

pp. 1116-1123 ◽

Cited By ~ 42

Author(s):

Xiaoming Liu ◽

Meihui Luo ◽

Liang N. Zhang ◽

Ziying Yan ◽

Roman Zak ◽

...

Keyword(s):

Cystic Fibrosis ◽

Epithelial Cells ◽

Airway Epithelial Cells ◽

Transmembrane Conductance Regulator ◽

Airway Epithelial ◽

Transmembrane Conductance ◽

Adeno Associated Virus ◽

Cystic Fibrosis Airway ◽

Cystic Fibrosis Transmembrane

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Up-regulation of AMP-activated Kinase by Dysfunctional Cystic Fibrosis Transmembrane Conductance Regulator in Cystic Fibrosis Airway Epithelial Cells Mitigates Excessive Inflammation

Journal of Biological Chemistry ◽

10.1074/jbc.m511029200 ◽

2006 ◽

Vol 281 (7) ◽

pp. 4231-4241 ◽

Cited By ~ 54

Author(s):

Kenneth R. Hallows ◽

Adam C. Fitch ◽

Christine A. Richardson ◽

Paul R. Reynolds ◽

John P. Clancy ◽

...

Keyword(s):

Cystic Fibrosis ◽

Epithelial Cells ◽

Airway Epithelial Cells ◽

Transmembrane Conductance Regulator ◽

Airway Epithelial ◽

Transmembrane Conductance ◽

Cystic Fibrosis Airway ◽

Cystic Fibrosis Transmembrane

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Spliceosome-Mediated RNA Trans-Splicing with Recombinant Adeno-Associated Virus Partially Restores Cystic Fibrosis Transmembrane Conductance Regulator Function to Polarized Human Cystic Fibrosis Airway Epithelial Cells

Human Gene Therapy ◽

10.1089/hum.2005.16.ft-107 ◽

2005 ◽

Vol 0 (0) ◽

pp. 050808081439001 ◽

Cited By ~ 1

Author(s):

Xiaoming Liu ◽

Meihui Luo ◽

Liang N. Zhang ◽

Ziying Yan ◽

Roman Zak ◽

...

Keyword(s):

Cystic Fibrosis ◽

Epithelial Cells ◽

Airway Epithelial Cells ◽

Transmembrane Conductance Regulator ◽

Airway Epithelial ◽

Transmembrane Conductance ◽

Adeno Associated Virus ◽

Cystic Fibrosis Airway ◽

Trans Splicing ◽

Cystic Fibrosis Transmembrane

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Normalization of raised sodium absorption and raised calcium-mediated chloride secretion by adenovirus-mediated expression of cystic fibrosis transmembrane conductance regulator in primary human cystic fibrosis airway epithelial cells.

Journal of Clinical Investigation ◽

10.1172/jci117789 ◽

1995 ◽

Vol 95 (3) ◽

pp. 1377-1382 ◽

Cited By ~ 123

Author(s):

L G Johnson ◽

S E Boyles ◽

J Wilson ◽

R C Boucher

Keyword(s):

Cystic Fibrosis ◽

Epithelial Cells ◽

Chloride Secretion ◽

Airway Epithelial Cells ◽

Sodium Absorption ◽

Transmembrane Conductance Regulator ◽

Airway Epithelial ◽

Transmembrane Conductance ◽

Cystic Fibrosis Airway ◽

Cystic Fibrosis Transmembrane

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Functional Cystic Fibrosis Transmembrane Conductance Regulator Expression in Cystic Fibrosis Airway Epithelial Cells by AAV6.2-Mediated SegmentalTrans-Splicing

Human Gene Therapy ◽

10.1089/hum.2008.173 ◽

2009 ◽

Vol 20 (3) ◽

pp. 267-281 ◽

Cited By ~ 34

Author(s):

Yuhu Song ◽

Howard H. Lou ◽

Julie L. Boyer ◽

Maria P. Limberis ◽

Luk H. Vandenberghe ◽

...

Keyword(s):

Cystic Fibrosis ◽

Epithelial Cells ◽

Airway Epithelial Cells ◽

Transmembrane Conductance Regulator ◽

Airway Epithelial ◽

Transmembrane Conductance ◽

Cystic Fibrosis Airway ◽

Cystic Fibrosis Transmembrane

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Faculty Opinions recommendation of Cystic fibrosis transmembrane conductance regulator is expressed in mucin granules from Calu-3 and primary human airway epithelial cells.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.718017856.793479176 ◽

2013 ◽

Author(s):

Steven M Rowe ◽

Sammeta Vamsee Raju

Keyword(s):

Cystic Fibrosis ◽

Epithelial Cells ◽

Airway Epithelial Cells ◽

Transmembrane Conductance Regulator ◽

Airway Epithelial ◽

Transmembrane Conductance ◽

Human Airway ◽

Cystic Fibrosis Transmembrane ◽

Human Airway Epithelial Cells

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Functional Stability of Rescued ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator in Airway Epithelial Cells

American Journal of Respiratory Cell and Molecular Biology ◽

10.1165/rcmb.2008-0434oc ◽

2010 ◽

Vol 42 (3) ◽

pp. 363-372 ◽

Cited By ~ 39

Author(s):

Asta Jurkuvenaite ◽

Lan Chen ◽

Rafal Bartoszewski ◽

Rebecca Goldstein ◽

Zsuzsa Bebok ◽

...

Keyword(s):

Cystic Fibrosis ◽

Epithelial Cells ◽

Airway Epithelial Cells ◽

Transmembrane Conductance Regulator ◽

Functional Stability ◽

Airway Epithelial ◽

Transmembrane Conductance ◽

Cystic Fibrosis Transmembrane

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Myosin Vb Is Required for Trafficking of the Cystic Fibrosis Transmembrane Conductance Regulator in Rab11a-specific Apical Recycling Endosomes in Polarized Human Airway Epithelial Cells

Journal of Biological Chemistry ◽

10.1074/jbc.m608531200 ◽

2007 ◽

Vol 282 (32) ◽

pp. 23725-23736 ◽

Cited By ~ 81

Author(s):

Agnieszka Swiatecka-Urban ◽

Laleh Talebian ◽

Eiko Kanno ◽

Sophie Moreau-Marquis ◽

Bonita Coutermarsh ◽

...

Keyword(s):

Cystic Fibrosis ◽

Epithelial Cells ◽

Airway Epithelial Cells ◽

Transmembrane Conductance Regulator ◽

Airway Epithelial ◽

Transmembrane Conductance ◽

Recycling Endosomes ◽

Human Airway ◽

Myosin Vb ◽

Cystic Fibrosis Transmembrane

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Turnover of the cystic fibrosis transmembrane conductance regulator (CFTR): Slow degradation of wild-type and ΔF508 CFTR in surface membrane preparations of immortalized airway epithelial cells

Journal of Cellular Physiology ◽

10.1002/(sici)1097-4652(199608)168:2<373::aid-jcp16>3.0.co;2-4 ◽

1996 ◽

Vol 168 (2) ◽

pp. 373-384 ◽

Cited By ~ 19

Author(s):

Xiaofang Wei ◽

Robin Eisman ◽

Jin Xu ◽

Alan D. Harsch ◽

Andrew E. Mulberg ◽

...

Keyword(s):

Cystic Fibrosis ◽

Epithelial Cells ◽

Surface Membrane ◽

Airway Epithelial Cells ◽

Transmembrane Conductance Regulator ◽

Airway Epithelial ◽

Wild Type ◽

Transmembrane Conductance ◽

Δf508 Cftr ◽

Cystic Fibrosis Transmembrane

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c-Cbl Facilitates Endocytosis and Lysosomal Degradation of Cystic Fibrosis Transmembrane Conductance Regulator in Human Airway Epithelial Cells

Journal of Biological Chemistry ◽

10.1074/jbc.m110.139881 ◽

2010 ◽

Vol 285 (35) ◽

pp. 27008-27018 ◽

Cited By ~ 36

Author(s):

Siying Ye ◽

Kristine Cihil ◽

Donna Beer Stolz ◽

Joseph M. Pilewski ◽

Bruce A. Stanton ◽

...

Keyword(s):

Cystic Fibrosis ◽

Epithelial Cells ◽

Airway Epithelial Cells ◽

Transmembrane Conductance Regulator ◽

Airway Epithelial ◽

Lysosomal Degradation ◽

Transmembrane Conductance ◽

Human Airway ◽

Cystic Fibrosis Transmembrane ◽

Human Airway Epithelial Cells

Download Full-text