Article
In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study.
BMC medicine - 29 Mar 2007
Sermet-Gaudelus Isabelle, Renouil Michel, Fajac Anne, Bidou Laure, Parbaille Bastien, Pierrot Sébastien, Davy Nolwen, Bismuth Elise, Reinert Philippe, Lenoir Gérard, Lesure Jean François, Rousset Jean Pierre, Edelman Aleksander
Abstract excerpt
BACKGROUND: Cystic fibrosis (CF) is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein, which acts as a chloride channel activated by cyclic AMP (cAMP). The most frequent mutation found in 70% of CF patients is F508del, while premature stop mutations are found in about 10% of patients. In vitro aminoglycoside antibiotics (e.g. gentamicin) suppress...
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