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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Bibliographic Details
Main Author: Matos, Ana M. (author)
Other Authors: Pinto, Francisco R. (author), Barros, Patrícia (author), Amaral, Margarida D. (author), Pepperkok, Rainer (author), Matos, Paulo (author)
Format: article
Language:eng
Published: 2020
Subjects:
Online Access:http://hdl.handle.net/10400.18/6529
Country:Portugal
Oai:oai:repositorio.insa.pt:10400.18/6529