Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Potentiator VX-770 (Ivacaftor) Opens the Defective Channel Gate of Mutant CFTR in a Phosphorylation-dependent but ATP-independent Manner

The cystic fibrosis transmembrane conductance regulator (CFTR) acts as a channel on the apical membrane of epithelia. Disease-causing mutations in the cystic fibrosis gene can lead to CFTR protein misfolding as in the case of the F508del mutation and/or channel dysfunction. Recently, a small molecul...

Πλήρης περιγραφή

Αποθηκεύτηκε σε:
Λεπτομέρειες βιβλιογραφικής εγγραφής
Κύριοι συγγραφείς: Eckford, Paul D. W., Li, Canhui, Ramjeesingh, Mohabir, Bear, Christine E.
Μορφή: Artigo
Γλώσσα:Inglês
Έκδοση: American Society for Biochemistry and Molecular Biology 2012
Θέματα:
Membrane Biology
Διαθέσιμο Online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3481266/
https://ncbi.nlm.nih.gov/pubmed/22942289
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M112.393637
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