Rescue of CFTR NBD2 mutants N1303K and S1235R is influenced by the functioning of the autophagosome

The missing phenylalanine at position 508, located in nucleotide-binding domain (NBD1) of the cystic fibrosis transmembrane regulator (CFTR), is the most common cystic fibrosis mutation. Severe disease-causing mutations also occur in NBD2. To provide information on potential therapeutic strategies f...

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Veröffentlicht in:	Journal of cystic fibrosis 2018-09, Vol.17 (5), p.582-594
Hauptverfasser:	Liu, Qiangni, Sabirzhanova, Inna, Yanda, Murali K., Bergbower, Emily A.S., Boinot, Clément, Guggino, William B., Cebotaru, Liudmila
Format:	Artikel
Sprache:	eng
Schlagworte:	Aminopyridines - pharmacology Animals Autophagosomes - physiology Autophagy Benzodioxoles - pharmacology Biological Transport Blotting, Western Cell Line CFTR Correctors Cystic Fibrosis - drug therapy Cystic Fibrosis - genetics Cystic Fibrosis Transmembrane Conductance Regulator - drug effects Cystic Fibrosis Transmembrane Conductance Regulator - genetics Degradation Electric Conductivity Leucine - analogs & derivatives Leucine - pharmacology Mutant Proteins - drug effects Mutant Proteins - genetics Mutation Mutations Short circuit current Small Molecule Libraries - pharmacology Western blot
Online-Zugang:	Volltext
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