Inhibition of calpain 1 restores plasma membrane stability to pharmacologically rescued Phe508del-CFTR variant

Cystic fibrosis (CF) is a genetic disease caused by mutations in the gene encoding CF transmembrane conductance regulator (CFTR), a chloride channel normally expressed at the surface of epithelial cells. The most frequent mutation, resulting in Phe-508 deletion, causes CFTR misfolding and its premat...

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Bibliografiske detaljer
Udgivet i:J Biol Chem
Main Authors: Matos, Ana M., Pinto, Francisco R., Barros, Patrícia, Amaral, Margarida D., Pepperkok, Rainer, Matos, Paulo
Format: Artigo
Sprog:Inglês
Udgivet: American Society for Biochemistry and Molecular Biology 2019
Fag:
Molecular Bases of Disease
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC6737230/
https://ncbi.nlm.nih.gov/pubmed/31324722
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.RA119.008738
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