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The major cystic fibrosis causing mutation exhibits defective propensity for phosphorylation

Proteomics (Weinheim), 2015-01, Vol.15 (2-3), p.447-461

2015

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Identification of binding sites for ivacaftor on the cystic fibrosis transmembrane conductance regulator

iScience, 2021-06, Vol.24 (6), p.102542-102542, Article 102542

2021

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Conformational defects underlie proteasomal degradation of Dent's disease-causing mutants of ClC-5

Biochemical journal, 2013-06, Vol.452 (3), p.391-400

2013

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Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein

Nature biotechnology, 2012-09, Vol.30 (9), p.876-882

2012

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The Chloride Channel ClC-4 Contributes to Endosomal Acidification and Trafficking

The Journal of biological chemistry, 2003-08, Vol.278 (31), p.29267-29277

2003

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The intact CFTR protein mediates ATPase rather than adenylate kinase activity

Biochemical journal, 2008-06, Vol.412 (2), p.315-321

2008

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Orkambi® and amplifier co‐therapy improves function from a rare CFTR mutation in gene‐edited cells and patient tissue

EMBO molecular medicine, 2017-09, Vol.9 (9), p.1224-1243

2017

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Comparison of a novel potentiator of CFTR channel activity to ivacaftor in ameliorating mucostasis caused by cigarette smoke in primary human bronchial airway epithelial cells

Respiratory research, 2024-07, Vol.25 (1), p.269-10, Article 269

2024

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ATP depletion inhibits the endocytosis of ClC-2

Journal of cellular physiology, 2008-01, Vol.214 (1), p.273-280

2008

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Novel method for evaluation of the oligomeric structure of membrane proteins

Biochemical journal, 1999-08, Vol.342 (1), p.119-123

1999

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The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF

Journal of cystic fibrosis, 2019-01, Vol.18 (1), p.35-43

2019

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A Heteromeric Complex of the Two Nucleotide Binding Domains of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mediates ATPase Activity

The Journal of biological chemistry, 2004-10, Vol.279 (40), p.41664-41669

2004

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Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise

Annals of neurology, 2010-06, Vol.67 (6), p.802-808

2010

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Walker Mutations Reveal Loose Relationship between Catalytic and Channel-Gating Activities of Purified CFTR (Cystic Fibrosis Transmembrane Conductance Regulator)

Biochemistry (Easton), 1999-02, Vol.38 (5), p.1463-1468

1999

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Perturbation of the Pore of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Inhibits Its ATPase Activity

The Journal of biological chemistry, 2001-04, Vol.276 (15), p.11575-11581

2001

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The Walker B motif of the second nucleotide-binding domain (NBD2) of CFTR plays a key role in ATPase activity by the NBD1-NBD2 heterodimer

Biochemical journal, 2007-01, Vol.401 (2), p.581-586

2007

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Nucleotides bind to the C-terminus of ClC-5

Biochemical journal, 2006-09, Vol.398 (2), p.289-294

2006

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A novel procedure for the efficient purification of the cystic fibrosis transmembrane conductance regulator (CFTR)

Biochemical journal, 1997-10, Vol.327 ( Pt 1) (1), p.17-21

1997

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A chemical corrector modifies the channel function of F508del-CFTR

Molecular pharmacology, 2010-09, Vol.78 (3), p.411-418

2010

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cDNA for the carboxyl-terminal region of a rat intestinal mucin-like peptide

The Journal of biological chemistry, 1992-03, Vol.267 (8), p.5401-5407

1992

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Novel method for evaluation of the oligomeric structure of membrane proteins

Biochemical journal, 1999-08, Vol.342 (1), p.119-123

1999

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Genetic, cell biological, and clinical interrogation of the CFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention

Genetics in medicine, 2014-08, Vol.16 (8), p.625-632

2014

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A Conserved Region of the R Domain of Cystic Fibrosis Transmembrane Conductance Regulator Is Important in Processing and Function

The Journal of biological chemistry, 1998-11, Vol.273 (48), p.31759-31764

1998

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Assessment of the efficacy of in vivo CFTR protein replacement therapy in CF mice

Human gene therapy, 1998-03, Vol.9 (4), p.521-528

1998

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Genetic, cell biological, and clinical interrogation of theCFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention

Genetics in medicine, 2014-08, Vol.16 (8), p.625-632

2014

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The major cystic fibrosis causing mutation exhibits defective propensity for phosphorylation

Identification of binding sites for ivacaftor on the cystic fibrosis transmembrane conductance regulator

Conformational defects underlie proteasomal degradation of Dent's disease-causing mutants of ClC-5

Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein

The Chloride Channel ClC-4 Contributes to Endosomal Acidification and Trafficking

The intact CFTR protein mediates ATPase rather than adenylate kinase activity

Orkambi® and amplifier co‐therapy improves function from a rare CFTR mutation in gene‐edited cells and patient tissue

Comparison of a novel potentiator of CFTR channel activity to ivacaftor in ameliorating mucostasis caused by cigarette smoke in primary human bronchial airway epithelial cells

ATP depletion inhibits the endocytosis of ClC-2

Novel method for evaluation of the oligomeric structure of membrane proteins

The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF

A Heteromeric Complex of the Two Nucleotide Binding Domains of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mediates ATPase Activity

Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise

Walker Mutations Reveal Loose Relationship between Catalytic and Channel-Gating Activities of Purified CFTR (Cystic Fibrosis Transmembrane Conductance Regulator)

Perturbation of the Pore of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Inhibits Its ATPase Activity

The Walker B motif of the second nucleotide-binding domain (NBD2) of CFTR plays a key role in ATPase activity by the NBD1-NBD2 heterodimer

Nucleotides bind to the C-terminus of ClC-5

A novel procedure for the efficient purification of the cystic fibrosis transmembrane conductance regulator (CFTR)

A chemical corrector modifies the channel function of F508del-CFTR

cDNA for the carboxyl-terminal region of a rat intestinal mucin-like peptide

Novel method for evaluation of the oligomeric structure of membrane proteins

Genetic, cell biological, and clinical interrogation of the CFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention

A Conserved Region of the R Domain of Cystic Fibrosis Transmembrane Conductance Regulator Is Important in Processing and Function

Assessment of the efficacy of in vivo CFTR protein replacement therapy in CF mice

Genetic, cell biological, and clinical interrogation of theCFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention

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