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Article

CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTR.

British journal of pharmacology - 1 Oct 2014

Wang Yiting, Liu Jia, Loizidou Avgi, Bugeja Luc A, Warner Ross, Hawley Bethan R, Cai Zhiwei, Toye Ashley M, Sheppard David N, Li Hongyu

Abstract excerpt

BACKGROUND AND PURPOSE: Dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl(-) channel causes the genetic disease cystic fibrosis (CF). Towards the development of transformational drug therapies for CF, we investigated the channel function and action of CFTR potentiators on A561E, a CF mutation found frequently in Portugal. Like the most common CF mutation F508del, A561E causes a...

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Identifiers

PMID: 24902474
DOI: 10.1111/bph.12791
PMCID: PMC4209154

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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