Small molecule correctors divert CFTR-F508del from ERAD by stabilizing sequential folding states

Over 80% of people with cystic fibrosis (CF) carry the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR), a chloride ion channel at the apical plasma membrane (PM) of epithelial cells. F508del impairs CFTR folding causing it to be destroyed by endoplasmic reticulum a...

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Detalles Bibliográficos
Autores principales: Riepe, Celeste, Wąchalska, Magda, Deol, Kirandeep K., Amaya, Anais K., Porteus, Matthew H., Olzmann, James A., Kopito, Ron R.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cold Spring Harbor Laboratory 2023
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10515913/
https://www.ncbi.nlm.nih.gov/pubmed/37745470
http://dx.doi.org/10.1101/2023.09.15.556420

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10515913/
https://www.ncbi.nlm.nih.gov/pubmed/37745470
http://dx.doi.org/10.1101/2023.09.15.556420

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