Ontology highlight
ABSTRACT:
SUBMITTER: Sinha C
PROVIDER: S-EPMC4672727 | biostudies-literature | 2015 Sep
REPOSITORIES: biostudies-literature
Sinha Chandrima C Zhang Weiqiang W Moon Chang Suk CS Actis Marcelo M Yarlagadda Sunitha S Arora Kavisha K Woodroofe Koryse K Clancy John P JP Lin Songbai S Ziady Assem G AG Frizzell Raymond R Fujii Naoaki N Naren Anjaparavanda P AP
Chembiochem : a European journal of chemical biology 20150811 14
Cystic fibrosis (CF) is a lethal genetic disease caused by the loss or dysfunction of the CF transmembrane conductance regulator (CFTR) channel. F508del is the most prevalent mutation of the CFTR gene and encodes a protein defective in folding and processing. VX-809 has been reported to facilitate the folding and trafficking of F508del-CFTR and augment its channel function. The mechanism of action of VX-809 has been poorly understood. In this study, we sought to answer a fundamental question und ...[more]