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...

Descripción completa

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

Internet

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

Ejemplares similares