Genetic Inhibition Of The Ubiquitin Ligase Rnf5 Attenuates Phenotypes Associated To F508del Cystic Fibrosis Mutation

Cystic fibrosis (CF) is caused by mutations in the CFTR chloride channel. Deletion of phenylalanine 508 (F508del), the most frequent CF mutation, impairs CFTR trafficking and gating. F508del-CFTR mistrafficking may be corrected by acting directly on mutant CFTR itself or by modulating expression/act...

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Detalles Bibliográficos
Autores principales: Tomati, Valeria, Sondo, Elvira, Armirotti, Andrea, Caci, Emanuela, Pesce, Emanuela, Marini, Monica, Gianotti, Ambra, Ju Jeon, Young, Cilli, Michele, Pistorio, Angela, Mastracci, Luca, Ravazzolo, Roberto, Scholte, Bob, Ronai, Ze’ev, Galietta, Luis J. V., Pedemonte, Nicoletta
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2015
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4505316/
https://www.ncbi.nlm.nih.gov/pubmed/26183966
http://dx.doi.org/10.1038/srep12138

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