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Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia

In cystic fibrosis (CF), deletion of phenylalanine 508 (F508del) in the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel causes misfolding and premature degradation. Considering the numerous effects of the F508del mutation on the assembly and processing of CFTR protein, combi...

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Bibliografiske detaljer
Udgivet i:JCI Insight
Main Authors: Tomati, Valeria, Caci, Emanuela, Ferrera, Loretta, Pesce, Emanuela, Sondo, Elvira, Cholon, Deborah M., Quinney, Nancy L., Boyles, Susan E., Armirotti, Andrea, Ravazzolo, Roberto, Galietta, Luis J.V., Gentzsch, Martina, Pedemonte, Nicoletta
Format: Artigo
Sprog:Inglês
Udgivet: American Society for Clinical Investigation 2018
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5821210/
https://ncbi.nlm.nih.gov/pubmed/29415893
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/jci.insight.98699
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