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Article

Ivacaftor potentiation of multiple CFTR channels with gating mutations.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 May 2012

Yu Haihui, Burton Bill, Huang Chien-Jung, Worley Jennings, Cao Dong, Johnson James P, Urrutia Art, Joubran John, Seepersaud Sheila, Sussky Katherine, Hoffman Beth J, Van Goor Fredrick

Abstract excerpt

BACKGROUND: The investigational CFTR potentiator ivacaftor (VX-770) increased CFTR channel activity and improved lung function in subjects with CF who have the G551D CFTR gating mutation. The aim of this in vitro study was to determine whether ivacaftor potentiates mutant CFTR with gating defects caused by other CFTR gating mutations. METHODS: The effects of ivacaftor on CFTR channel open probability and chloride...

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Source

Identifiers

PMID: 22293084
DOI: 10.1016/j.jcf.2011.12.005

Genes

CFTR

Variants

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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