VX-770-mediated potentiation of numerous human CFTR disease mutants is influenced by phosphorylation level

VX-770 (ivacaftor) is approved for clinical use in CF patients bearing multiple CFTR mutations. VX-770 potentiated wildtype CFTR and several disease mutants expressed in oocytes in a manner modulated by PKA-mediated phosphorylation. Potentiation of some other mutants, including G551D-CFTR, was less...

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Detalles Bibliográficos
Autores principales: Cui, Guiying, Stauffer, Brandon B., Imhoff, Barry R., Rab, Andras, Hong, Jeong S., Sorscher, Eric J., McCarty, Nael A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2019
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6749054/
https://www.ncbi.nlm.nih.gov/pubmed/31530897
http://dx.doi.org/10.1038/s41598-019-49921-4

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6749054/
https://www.ncbi.nlm.nih.gov/pubmed/31530897
http://dx.doi.org/10.1038/s41598-019-49921-4

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