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Additive effect of multiple pharmacological chaperones on maturation of CFTR processing mutants

The most common cause of CF (cystic fibrosis) is the deletion of Phe(508) (ΔF508) in the CFTR [CF TM (transmembrane) conductance regulator] chloride channel. One major problem with ΔF508 CFTR is that the protein is defective in folding so that little mature protein is delivered to the cell surface....

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Détails bibliographiques
Auteurs principaux: Wang, Ying, Loo, Tip W., Bartlett, M. Claire, Clarke, David M.
Format: Artigo
Langue:Inglês
Publié: Portland Press Ltd. 2007
Sujets:
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC1948964/
https://ncbi.nlm.nih.gov/pubmed/17535157
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1042/BJ20070478
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