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Characterization and Dynamics of Aggresome Formation by a Cytosolic GFP-Chimera

The Journal of cell biology, 1999-09, Vol.146 (6), p.1239-1254

1999

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A clinical inflammatory syndrome attributable to aerosolized lipid-DNA administration in cystic fibrosis

Human gene therapy, 2001-05, Vol.12 (7), p.751-761

2001

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Gentamicin-Induced Correction of CFTR Function in Patients with Cystic Fibrosis and CFTR Stop Mutations

The New England journal of medicine, 2003-10, Vol.349 (15), p.1433-1441

2003

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Failure of cAMP agonists to activate rescued ΔF508 CFTR in CFBE41o– airway epithelial monolayers

The Journal of physiology, 2005-12, Vol.569 (2), p.601-615

2005

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Enhanced cell-surface stability of rescued ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones

Biochemical journal, 2008-03, Vol.410 (3), p.555-564

2008

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Improved oxygenation promotes CFTR maturation and trafficking in MDCK monolayers

American Journal of Physiology: Cell Physiology, 2001-01, Vol.280 (1), p.C135-C145

2001

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Evidence that Systemic Gentamicin Suppresses Premature Stop Mutations in Patients with Cystic Fibrosis

American journal of respiratory and critical care medicine, 2001-06, Vol.163 (7), p.1683-1692

2001

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Efficient Intracellular Processing of the Endogenous Cystic Fibrosis Transmembrane Conductance Regulator in Epithelial Cell Lines

The Journal of biological chemistry, 2004-05, Vol.279 (21), p.22578-22584

2004

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Extracellular Zinc and ATP Restore Chloride Secretion across Cystic Fibrosis Airway Epithelia by Triggering Calcium Entry

The Journal of biological chemistry, 2004-03, Vol.279 (11), p.10720-10729

2004

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Mutations in the Amino Terminus of the Cystic Fibrosis Transmembrane Conductance Regulator Enhance Endocytosis

The Journal of biological chemistry, 2006-02, Vol.281 (6), p.3329-3334

2006

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Reactive oxygen nitrogen species decrease cystic fibrosis transmembrane conductance regulator expression and cAMP-mediated Cl- secretion in airway epithelia

The Journal of biological chemistry, 2002-11, Vol.277 (45), p.43041-43049

2002

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Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line

Nature medicine, 1997-11, Vol.3 (11), p.1280-1284

1997

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Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene

Journal of molecular medicine (Berlin, Germany), 2002-09, Vol.80 (9), p.595-604

2002

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Excellent in vivo bystander activity of fludarabine phosphate against human glioma xenografts that express the Escherichia coli purine nucleoside phosphorylase gene

Cancer research (Chicago, Ill.), 2004-09, Vol.64 (18), p.6610-6615

2004

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Novel regulation of cell [Na(+)] in macula densa cells: apical Na(+) recycling by H-K-ATPase

American journal of physiology. Renal physiology, 2002-02, Vol.282 (2), p.F324-F329

2002

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Macula densa Na(+)/H(+) exchange activities mediated by apical NHE2 and basolateral NHE4 isoforms

American journal of physiology. Renal physiology, 2000-03, Vol.278 (3), p.F452-F463

2000

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Ablation of Internalization Signals in the Carboxyl-terminal Tail of the Cystic Fibrosis Transmembrane Conductance Regulator Enhances Cell Surface Expression

The Journal of biological chemistry, 2002-12, Vol.277 (51), p.49952-49957

2002

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A method for the rapid detection of recombinant CFTR during gene therapy in cystic fibrosis

Gene therapy, 1996-08, Vol.3 (8), p.685

1996

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The Mechanism Underlying Cystic Fibrosis Transmembrane Conductance Regulator Transport from the Endoplasmic Reticulum to the Proteasome Includes Sec61β and a Cytosolic, Deglycosylated Intermediary

The Journal of biological chemistry, 1998-11, Vol.273 (45), p.29873-29878

1998

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Transient transfection of polarized epithelial monolayers with CFTR and reporter genes using efficacious lipids

American Journal of Physiology: Cell Physiology, 2003-03, Vol.284 (3), p.C791-C804

2003

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Tumor cell bystander killing in colonic carcinoma utilizing the Escherichia coli DeoD gene to generate toxic purines

Gene therapy, 1994-07, Vol.1 (4), p.233

1994

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Establishment and Characterization of a Novel Polarized MDCK Epithelial Cellular Model for CFTR Studies

Cellular physiology and biochemistry, 2005-01, Vol.16 (4-6), p.281-290

2005

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Purification, characterization, and expression of CFTR nucleotide-binding domains

Journal of bioenergetics and biomembranes, 1997-10, Vol.29 (5), p.475-482

1997

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Consequences of C-terminal domains and N-terminal signal peptide deletions on LEKTI secretion, stability, and subcellular distribution

Archives of biochemistry and biophysics, 2005-03, Vol.435 (1), p.89-102

2005

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Activation of Delta F508 CFTR in an epithelial monolayer

American Journal of Physiology: Cell Physiology, 1998-08, Vol.275 (2), p.C599-C607

1998

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Characterization and Dynamics of Aggresome Formation by a Cytosolic GFP-Chimera

A clinical inflammatory syndrome attributable to aerosolized lipid-DNA administration in cystic fibrosis

Gentamicin-Induced Correction of CFTR Function in Patients with Cystic Fibrosis and CFTR Stop Mutations

Failure of cAMP agonists to activate rescued ΔF508 CFTR in CFBE41o– airway epithelial monolayers

Enhanced cell-surface stability of rescued ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones

Improved oxygenation promotes CFTR maturation and trafficking in MDCK monolayers

Evidence that Systemic Gentamicin Suppresses Premature Stop Mutations in Patients with Cystic Fibrosis

Efficient Intracellular Processing of the Endogenous Cystic Fibrosis Transmembrane Conductance Regulator in Epithelial Cell Lines

Extracellular Zinc and ATP Restore Chloride Secretion across Cystic Fibrosis Airway Epithelia by Triggering Calcium Entry

Mutations in the Amino Terminus of the Cystic Fibrosis Transmembrane Conductance Regulator Enhance Endocytosis

Reactive oxygen nitrogen species decrease cystic fibrosis transmembrane conductance regulator expression and cAMP-mediated Cl- secretion in airway epithelia

Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line

Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene

Excellent in vivo bystander activity of fludarabine phosphate against human glioma xenografts that express the Escherichia coli purine nucleoside phosphorylase gene

Novel regulation of cell [Na(+)] in macula densa cells: apical Na(+) recycling by H-K-ATPase

Macula densa Na(+)/H(+) exchange activities mediated by apical NHE2 and basolateral NHE4 isoforms

Ablation of Internalization Signals in the Carboxyl-terminal Tail of the Cystic Fibrosis Transmembrane Conductance Regulator Enhances Cell Surface Expression

A method for the rapid detection of recombinant CFTR during gene therapy in cystic fibrosis

The Mechanism Underlying Cystic Fibrosis Transmembrane Conductance Regulator Transport from the Endoplasmic Reticulum to the Proteasome Includes Sec61β and a Cytosolic, Deglycosylated Intermediary

Transient transfection of polarized epithelial monolayers with CFTR and reporter genes using efficacious lipids

Tumor cell bystander killing in colonic carcinoma utilizing the Escherichia coli DeoD gene to generate toxic purines

Establishment and Characterization of a Novel Polarized MDCK Epithelial Cellular Model for CFTR Studies

Purification, characterization, and expression of CFTR nucleotide-binding domains

Consequences of C-terminal domains and N-terminal signal peptide deletions on LEKTI secretion, stability, and subcellular distribution

Activation of Delta F508 CFTR in an epithelial monolayer

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