Research Topics
Species | R C RubensteinSummaryAffiliation: Johns Hopkins University Country: USA Publications
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Publications
In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTRR C Rubenstein
Eudowood Division of Pediatric Respiratory Sciences, Johns Hopkins Hospital, Baltimore, Maryland 21287, USA
J Clin Invest 100:2457-65. 1997..These data indicate that 4PBA is a promising pharmacologic agent for inducing correction of the CF phenotype in CF patients carrying the delta F508 mutation...
A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR functionR C Rubenstein
Eudowood Division of Pediatric Respiratory Sciences, The Johns Hopkins Medical Institutions, Baltimore, Maryland 21287, USA
Am J Respir Crit Care Med 157:484-90. 1998..Side effects due to drug therapy were minimal and comparable in the two groups. These data are consistent with 4PBA therapy inducing CFTR function in the nasal epithelia of deltaF508-homozygous CF patients...
Mutation in the gene responsible for cystic fibrosis and predisposition to chronic rhinosinusitis in the general populationX Wang
McKusick Nathans Institute of Genetic Medicine, CMSC 1004, Baltimore, MD 21287, USA
JAMA 284:1814-9. 2000..Chronic rhinosinusitis is a consistent feature of the autosomal recessive disorder cystic fibrosis (CF)...
