Cargando…
Combination of Correctors Rescues CFTR Transmembrane-Domain Mutants by Mitigating their Interactions with Proteostasis
BACKGROUND/AIMS: Premature degradation of mutated cystic fibrosis transmembrane conductance regulator (CFTR) protein causes cystic fibrosis (CF), the commonest Mendelian disease in Caucasians. Despite recent advances in precision medicines for CF patients, many CFTR mutants have not been characteriz...
Autores principales: | Lopes-Pacheco, Miquéias, Boinot, Clément, Sabirzhanova, Inna, Rapino, Daniele, Cebotaru, Liudmila |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
2017
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7082854/ https://www.ncbi.nlm.nih.gov/pubmed/28448979 http://dx.doi.org/10.1159/000475578 |
Ejemplares similares
-
Rescue of NBD2 Mutants N1303K and S1235R of CFTR by Small-Molecule Correctors and Transcomplementation
por: Rapino, Daniele, et al.
Publicado: (2015) -
Syntaxin 8 and the Endoplasmic Reticulum Processing of ΔF508-CFTR
por: Sabirzhanova, Inna, et al.
Publicado: (2018) -
Correcting the Cystic Fibrosis Disease Mutant, A455E CFTR
por: Cebotaru, Liudmila, et al.
Publicado: (2014) -
Therapeutic Potential for CFTR Correctors in Autosomal Recessive Polycystic Kidney Disease
por: Yanda, Murali K., et al.
Publicado: (2021) -
Bithiazole Correctors Rescue CFTR Mutants by Two Different
Mechanisms
por: Loo, Tip W., et al.
Publicado: (2013)