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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Bibliographic Details
Published in: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
Language:Inglês
Published: MDPI 2021
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Online Access: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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