A Little CFTR Goes a Long Way: CFTR-Dependent Sweat Secretion from G551D and R117H-5T Cystic Fibrosis Subjects Taking Ivacaftor

To determine if oral dosing with the CFTR-potentiator ivacaftor (VX-770, Kalydeco) improves CFTR-dependent sweating in CF subjects carrying G551D or R117H-5T mutations, we optically measured sweat secretion from 32–143 individually identified glands in each of 8 CF subjects; 6 F508del/G551D, one G55...

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Detalles Bibliográficos
Autores principales: Char, Jessica E., Wolfe, Marlene H., Cho, Hyung-ju, Park, Il-Ho, Jeong, Jin Hyeok, Frisbee, Eric, Dunn, Colleen, Davies, Zoe, Milla, Carlos, Moss, Richard B., Thomas, Ewart A. C., Wine, Jeffrey J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2014
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3919757/
https://www.ncbi.nlm.nih.gov/pubmed/24520399
http://dx.doi.org/10.1371/journal.pone.0088564

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