The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) potentiator ivacaftor (Kalydeco®) improves clinical outcome in G551D cystic fibrosis (CF) patients. Here, we have investigated whether ivacaftor has a clinical impact on non-G551D gating mutations and function of circulating leukocytes as well.
CFTR-dependent chloride efflux in cystic fibrosis mononuclear cells is increased by ivacaftor therapy
Guerra, L.;Favia, M.;Castellani, S.;Polizzi, A. M.;Casavola, V.;Manca, A.;Conese, M.
2017-01-01
Abstract
The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) potentiator ivacaftor (Kalydeco®) improves clinical outcome in G551D cystic fibrosis (CF) patients. Here, we have investigated whether ivacaftor has a clinical impact on non-G551D gating mutations and function of circulating leukocytes as well.File in questo prodotto:
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