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Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Potentiators Protect G551D but Not ΔF508 CFTR from Thermal Instability

[Image: see text] The G551D cystic fibrosis transmembrane conductance regulator (CFTR) mutation is associated with severe disease in ∼5% of cystic fibrosis patients worldwide. This amino acid substitution in NBD1 results in a CFTR chloride channel characterized by a severe gating defect that can be...

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Detalles Bibliográficos
Autores principales:	Liu, Xuehong, Dawson, David C.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	American Chemical Society 2014
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4159205/ https://www.ncbi.nlm.nih.gov/pubmed/25148434 http://dx.doi.org/10.1021/bi501007v

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