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Distinct proteostasis states drive pharmacologic chaperone susceptibility for cystic fibrosis transmembrane conductance regulator misfolding mutants

Pharmacological chaperones represent a class of therapeutic compounds for treating protein misfolding diseases. One of the most prominent examples is the FDA-approved pharmacological chaperone lumacaftor (VX-809), which has transformed cystic fibrosis (CF) therapy. CF is a fatal disease caused by mu...

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Detalles Bibliográficos
Autores principales:	McDonald, Eli Fritz, Sabusap, Carleen Mae P., Kim, Minsoo, Plate, Lars
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	The American Society for Cell Biology 2022
Materias:	Articles
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9561855/ https://www.ncbi.nlm.nih.gov/pubmed/35389766 http://dx.doi.org/10.1091/mbc.E21-11-0578

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