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Article

The magnitude of ivacaftor effects on fluid secretion via R117H-CFTR channels: Human in vivo measurements.

PloS one - 1 Jan 2017

Char Jessica E, Dunn Colleen, Davies Zoe, Milla Carlos, Moss Richard B, Wine Jeffrey J

Abstract excerpt

We optically measured effects of orally available ivacaftor (Kalydeco®) on sweat rates of identified glands in 3 R117H subjects, each having a unique set of additional mutations, and compared them with 5 healthy control subjects tested contemporaneously. We injected β-adrenergic agonists intradermally to stimulate CFTR-dependent 'C-sweat' and methacholine to stimulate 'M-sweat', which persists in CF subjects. We...

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Identifiers

PMID: 28419121
DOI: 10.1371/journal.pone.0175486
PMCID: PMC5395152

Genes

CFTR

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