Cargando…
Treatment of Polarized Cystic Fibrosis Airway Cells With HGF Prevents VX-661-Rescued F508del-CFTR Destabilization Caused by Prolonged Co-exposure to VX-770
Cystic fibrosis (CF), the most common inherited disease in Caucasians, is caused by mutations in CFTR, the most frequent of which is F508del. F508del causes ER retention and degradation of the mutant CFTR protein, but also defective channel gating and decreased half-life at the plasma membrane. Desp...
Autores principales: | Matos, Ana M., Jordan, Peter, Matos, Paulo |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2021
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8727755/ https://www.ncbi.nlm.nih.gov/pubmed/35004859 http://dx.doi.org/10.3389/fmolb.2021.812101 |
Ejemplares similares
-
Identifying the molecular target sites for CFTR potentiators GLPG1837
and VX-770
por: Yeh, Han-I, et al.
Publicado: (2019) -
VX-770-mediated potentiation of numerous human CFTR disease mutants is influenced by phosphorylation level
por: Cui, Guiying, et al.
Publicado: (2019) -
Prolonged co-treatment with HGF sustains epithelial integrity and improves pharmacological rescue of Phe508del-CFTR
por: Matos, Ana M., et al.
Publicado: (2018) -
Theratyping of the Rare CFTR Genotype A559T in Rectal Organoids and Nasal Cells Reveals a Relevant Response to Elexacaftor (VX-445) and Tezacaftor (VX-661) Combination
por: Kleinfelder, Karina, et al.
Publicado: (2023) -
Pseudomonas aeruginosa Reduces VX-809 Stimulated F508del-CFTR Chloride Secretion by Airway Epithelial Cells
por: Stanton, Bruce A., et al.
Publicado: (2015)