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Ivacaftor treatment of cystic fibrosis patients with the G551D mutation: a review of the evidence

Cystic fibrosis (CF) is a recessive disorder caused by mutations in the gene that encodes the CF transmembrane conductance regulator (CFTR) protein. CFTR protein is a chloride and bicarbonate channel that is critical for normal epithelial ion transport and hydration of epithelial surfaces. Current C...

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Autors principals: Kavitha Kotha, John P. Clancy
Format: Artigo
Idioma:Inglês
Publicat: SAGE Publishing 2013-10-01
Col·lecció:Therapeutic Advances in Respiratory Disease
Accés en línia:https://doi.org/10.1177/1753465813502115
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