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Phase II studies of nebulised Arikace in CF patients with Pseudomonas aeruginosa infection

Thorax, 2013-09, Vol.68 (9), p.818-825

2013

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Long-term treatment with oral N-acetylcysteine: Affects lung function but not sputum inflammation in cystic fibrosis subjects. A phase II randomized placebo-controlled trial

Journal of cystic fibrosis, 2015-03, Vol.14 (2), p.219-227

2015

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A pilot clinical trial of oral sodium 4-phenylbutyrate (buphenyl) in ΔF508-homozygous cystic fibrosis patients: Partial restoration of nasal epithelial CFTR function

American journal of respiratory and critical care medicine, 1998-02, Vol.157 (2), p.484-490

1998

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Mutation in the gene responsible for cystic fibrosis and predisposition to chronic rhinosinusitis in the general population

JAMA : the journal of the American Medical Association, 2000-10, Vol.284 (14), p.1814

2000

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In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR

The Journal of clinical investigation, 1997-11, Vol.100 (10), p.2457-2465

1997

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CFTR gene transduction in neonatal rabbits using an adeno-associated virus (AAV) vector

Gene therapy, 1997-05, Vol.4 (5), p.384-392

1997

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Sodium 4-phenylbutyrate downregulates HSC70 expression by facilitating mRNA degradation

American journal of physiology. Lung cellular and molecular physiology, 2001-07, Vol.281 (1), p.43-L51

2001

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Acute flank pain and reversible renal dysfunction associated with nonsteroidal anti-inflammatory drug use

Pediatrics (Evanston), 1993-09, Vol.92 (3), p.459

1993

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D25 PEDIATRIC CYSTIC FIBROSIS: Regulation Of The Erp29 Promoter By The F508del-Cftr Corrector 4-Phenylbutyrate And The E2f-1 Tanscription Factor

American journal of respiratory and critical care medicine, 2014-01, Vol.189, p.1

2014

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The hydrophobic tryptic core of the beta-adrenergic receptor retains Gs regulatory activity in response to agonists and thiols

The Journal of biological chemistry, 1987-12, Vol.262 (34), p.16655-16662

1987

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Genistein Restores Functional Interactions between ΔF508-CFTR and ENaC in Xenopus Oocytes

The Journal of biological chemistry, 2002-03, Vol.277 (11), p.8928-8933

2002

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A topological data analytic approach for discovering biophysical signatures in protein dynamics

PLoS computational biology, 2022-05, Vol.18 (5), p.e1010045-e1010045

2022

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C62 PEDIATRIC CASES I: A Neonatal Presentation Of Co-Morbid Maple Syrup Urine Disease And Cystic Fibrosis

American journal of respiratory and critical care medicine, 2016-01, Vol.193, p.1

2016

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Genistein Improves Regulatory Interactions between G551D-Cystic Fibrosis Transmembrane Conductance Regulator and the Epithelial Sodium Channel in Xenopus Oocytes

The Journal of biological chemistry, 2002-12, Vol.277 (52), p.50341-50347

2002

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Nocturnal saturation and glucose tolerance in children with cystic fibrosis

Thorax, 2011-07, Vol.66 (7), p.574-578

2011

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Differential Effects of Hsc70 and Hsp70 on the Intracellular Trafficking and Functional Expression of Epithelial Sodium Channels

Proceedings of the National Academy of Sciences - PNAS, 2006-04, Vol.103 (15), p.5817-5822

2006

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Risk Factors for Death of Patients with Cystic Fibrosis Awaiting Lung Transplantation

American journal of respiratory and critical care medicine, 2006-03, Vol.173 (6), p.659-666

2006

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Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of Delta F508-CFTR

American Journal of Physiology: Cell Physiology, 2000-02, Vol.278 (2), p.C259-C267

2000

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A simplified cyclic adenosine monophosphate–mediated sweat rate test for quantitative measure of cystic fibrosis transmembrane regulator (CFTR) function

The Journal of pediatrics, 2000-12, Vol.137 (6), p.849-855

2000

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Evidence of CFTR Function in Cystic Fibrosis after Systemic Administration of 4-Phenylbutyrate

Molecular therapy, 2002-07, Vol.6 (1), p.119-126

2002

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Cystic Fibrosis Transmembrane Conductance Regulator Differentially Regulates Human and Mouse Epithelial Sodium Channels in Xenopus Oocytes

The Journal of biological chemistry, 2004-05, Vol.279 (22), p.23183-23192

2004

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Functional domains in the beta-adrenergic receptor

Cold Spring Harbor Symposia on Quantitative Biology, 1988, Vol.53 Pt 1, p.499

1988

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Novel, mechanism-based therapies for cystic fibrosis

Current opinion in pediatrics, 2005-06, Vol.17 (3), p.385-392

Rubenstein, Ronald C

2005

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Functional polymorphism in the carboxyl terminus of the alpha-subunit of the human epithelial sodium channel

The Journal of biological chemistry, 2004-06, Vol.279 (23), p.23900-23907

2004

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Differential modulation of a polymorphism in the COOH terminus of the α-subunit of the human epithelial sodium channel by protein kinase Cδ

American journal of physiology. Renal physiology, 2006-02, Vol.290 (2), p.F279-F288

2006

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Phase II studies of nebulised Arikace in CF patients with Pseudomonas aeruginosa infection

Long-term treatment with oral N-acetylcysteine: Affects lung function but not sputum inflammation in cystic fibrosis subjects. A phase II randomized placebo-controlled trial

A pilot clinical trial of oral sodium 4-phenylbutyrate (buphenyl) in ΔF508-homozygous cystic fibrosis patients: Partial restoration of nasal epithelial CFTR function

Mutation in the gene responsible for cystic fibrosis and predisposition to chronic rhinosinusitis in the general population

In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR

CFTR gene transduction in neonatal rabbits using an adeno-associated virus (AAV) vector

Sodium 4-phenylbutyrate downregulates HSC70 expression by facilitating mRNA degradation

Acute flank pain and reversible renal dysfunction associated with nonsteroidal anti-inflammatory drug use

D25 PEDIATRIC CYSTIC FIBROSIS: Regulation Of The Erp29 Promoter By The F508del-Cftr Corrector 4-Phenylbutyrate And The E2f-1 Tanscription Factor

The hydrophobic tryptic core of the beta-adrenergic receptor retains Gs regulatory activity in response to agonists and thiols

Genistein Restores Functional Interactions between ΔF508-CFTR and ENaC in Xenopus Oocytes

A topological data analytic approach for discovering biophysical signatures in protein dynamics

C62 PEDIATRIC CASES I: A Neonatal Presentation Of Co-Morbid Maple Syrup Urine Disease And Cystic Fibrosis

Genistein Improves Regulatory Interactions between G551D-Cystic Fibrosis Transmembrane Conductance Regulator and the Epithelial Sodium Channel in Xenopus Oocytes

Nocturnal saturation and glucose tolerance in children with cystic fibrosis

Differential Effects of Hsc70 and Hsp70 on the Intracellular Trafficking and Functional Expression of Epithelial Sodium Channels

Risk Factors for Death of Patients with Cystic Fibrosis Awaiting Lung Transplantation

Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of Delta F508-CFTR

A simplified cyclic adenosine monophosphate–mediated sweat rate test for quantitative measure of cystic fibrosis transmembrane regulator (CFTR) function

Evidence of CFTR Function in Cystic Fibrosis after Systemic Administration of 4-Phenylbutyrate

Cystic Fibrosis Transmembrane Conductance Regulator Differentially Regulates Human and Mouse Epithelial Sodium Channels in Xenopus Oocytes

Functional domains in the beta-adrenergic receptor

Novel, mechanism-based therapies for cystic fibrosis

Functional polymorphism in the carboxyl terminus of the alpha-subunit of the human epithelial sodium channel

Differential modulation of a polymorphism in the COOH terminus of the α-subunit of the human epithelial sodium channel by protein kinase Cδ

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