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

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...

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Main Authors: Wang, Yiting, Liu, Jia, Loizidou, Avgi, Bugeja, Luc A, Warner, Ross, Hawley, Bethan R, Cai, Zhiwei, Toye, Ashley M, Sheppard, David N, Li, Hongyu
Formato: Artigo
Idioma:Inglês
Publicado: BlackWell Publishing Ltd 2014
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Research Papers
Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC4209154/
https://ncbi.nlm.nih.gov/pubmed/24902474
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1111/bph.12791
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