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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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Salvato in:
Dettagli Bibliografici
Pubblicato in:Sci Rep
Autori principali: 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
Natura: Artigo
Lingua:Inglês
Pubblicazione: Nature Publishing Group 2015
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC4505316/
https://ncbi.nlm.nih.gov/pubmed/26183966
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/srep12138
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