Article
Correctors Rescue CFTR Mutations in Nucleotide-Binding Domain 1 (NBD1) by Modulating Proteostasis.
Chembiochem : a European journal of chemical biology - 15 Mar 2016
Lopes-Pacheco Miquéias, Sabirzhanova Inna, Rapino Daniele, Morales Marcelo M, Guggino William B, Cebotaru Liudmila
Abstract excerpt
We evaluated whether small molecule correctors could rescue four nucleotide-binding domain 1 (NBD1) mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene (A455E, S492F, ΔI507, and R560T). We first transfected Cos-7 cells (green monkey kidney cells) with A455E, S492F, ΔI507, or R560T and created HEK-293 (human embryonic kidney cells) cell lines stably expressing these CFTR mutations. The...
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