Ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation
Cystic fibrosis (CF) is an autosomal recessive lethal disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that encodes for CFTR, an epithelial cell-surface expressed protein responsible for the transport of chloride (Cl-). Gating mutations associated wi...
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| Formatua: | Artigo |
| Hizkuntza: | Inglês |
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European Respiratory Society
2013-03-01
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| Saila: | European Respiratory Review |
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| Sarrera elektronikoa: | http://err.ersjournals.com/content/22/127/66.full.pdf+html |
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