Activity of lumacaftor is not conserved in zebrafish Cftr bearing the major cystic fibrosis‐causing mutation

Activity of lumacaftor is not conserved in zebrafish Cftr bearing the major cystic fibrosis‐causing mutation

Abstract F508del‐cystic fibrosis transmembrane conductance regulator (CFTR) is the major mutant responsible for cystic fibrosis (CF). ORKAMBI®, approved for patients bearing this mutant, contains lumacaftor (VX‐809) that partially corrects F508del‐CFTR's processing defect and ivacaftor (VX‐770)...

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Bibliographic Details
Main Authors: Onofrio Laselva, Steven Erwood, Kai Du, Zhenya Ivakine, Christine E. Bear
Format: Article
Language:English
Published: Wiley 2019-10-01
Series:FASEB BioAdvances
Subjects:
ABC transporters
CFTR
CFTR modulators
cystic fibrosis
F508del‐CFTR
zebrafish
Online Access:https://doi.org/10.1096/fba.2019-00039

Internet

https://doi.org/10.1096/fba.2019-00039

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