Formulation: A solid
Formal Name: N-[2,4-bis[1,1-di(methyl-d3)ethyl-2,2,2-d3]-5-hydroxyphenyl-3d]-1,4-dihydro-4-oxo-3-quinolinecarboxamide
Purity: ≥99% deuterated forms (d1-d19)
Formula Markup: C24H9D19N2O3
Formula Weight: 411,6
Shelf life (days): 1460
CAS Number: 1413431-22-7
Notes: Ivacaftor-d19 is intended for use as an internal standard for the quantification of ivacaftor (Item No. 15145) by GC- or LC-MS. 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 ΔF508 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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