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

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) that p...

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Detalles Bibliográficos
Autores principales: Laselva, Onofrio, Erwood, Steven, Du, Kai, Ivakine, Zhenya, Bear, Christine E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2019
Materias:
Research Articles
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6996396/
https://www.ncbi.nlm.nih.gov/pubmed/32123813
http://dx.doi.org/10.1096/fba.2019-00039

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6996396/
https://www.ncbi.nlm.nih.gov/pubmed/32123813
http://dx.doi.org/10.1096/fba.2019-00039

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