Ivacaftor

Ivacaftor

CAT N°: 15145
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From 49.00 41.65

Ivacaftor is an orally bioavailable potentiator of the cystic fibrosis transmembrane conductance regulator (CFTR) that improves chloride transport.{25614} It increases the forskolin-induced CFTR-mediated epithelial current in cells expressing the G551D missense mutation associated with severe cystic fibrosis by approximately 4-fold (EC50 = 100 nM) but has no effect on current in the absence of forskolin.{25614} Ivacaftor increases chloride secretion in cultured human cystic fibrosis bronchial epithelial cells carrying the G551D mutation on one allele and the common ?F508 processing mutation on the other allele.{25614} It binds CFTR directly and leads to CFTR channel opening via an ATP-independent mechanism.{25613} Formulations containing ivacaftor have been used in the treatment of cystic fibrosis in patients carrying one or more mutations in the CFTR gene.

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