Partial Rescue of F508del-CFTR Stability and Trafficking Defects by Double Corrector Treatment

Deletion of phenylalanine at position 508 (F508del) in the CFTR chloride channel is the most frequent mutation in cystic fibrosis (CF) patients. F508del impairs the stability and folding of the CFTR protein, thus resulting in mistrafficking and premature degradation. F508del-CFTR defects can be over...

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Bibliografske podrobnosti
izdano v:Int J Mol Sci
Main Authors: Capurro, Valeria, Tomati, Valeria, Sondo, Elvira, Renda, Mario, Borrelli, Anna, Pastorino, Cristina, Guidone, Daniela, Venturini, Arianna, Giraudo, Alessandro, Mandrup Bertozzi, Sine, Musante, Ilaria, Bertozzi, Fabio, Bandiera, Tiziano, Zara, Federico, Galietta, Luis J. V., Pedemonte, Nicoletta
Format: Artigo
Jezik:Inglês
Izdano: MDPI 2021
Teme:
Article
Online dostop:https://ncbi.nlm.nih.gov/pmc/articles/PMC8156943/
https://ncbi.nlm.nih.gov/pubmed/34067708
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3390/ijms22105262
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