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Folding–function relationship of the most common cystic fibrosis–causing CFTR conductance mutants

Cystic fibrosis is caused by mutations in the CFTR gene, which are subdivided into six classes. Mutants of classes III and IV reach the cell surface but have limited function. Most class-III and class-IV mutants respond well to the recently approved potentiator VX-770, which opens the channel. We he...

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Detalles Bibliográficos
Autores principales: van Willigen, Marcel, Vonk, Annelotte M, Yeoh, Hui Ying, Kruisselbrink, Evelien, Kleizen, Bertrand, van der Ent, Cornelis K, Egmond, Maarten R, de Jonge, Hugo R, Braakman, Ineke, Beekman, Jeffrey M, van der Sluijs, Peter
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Life Science Alliance LLC 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6339265/
https://www.ncbi.nlm.nih.gov/pubmed/30659068
http://dx.doi.org/10.26508/lsa.201800172