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Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia

Pulmonary disease is the major cause of morbidity and mortality in patients with cystic fibrosis, a disease caused by mutations in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene. Heterogeneity in CFTR genotype–phenotype relationships in affected individuals plus the escalation o...

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Detalles Bibliográficos
Autores principales: Ahmadi, Saumel, Bozoky, Zoltan, Di Paola, Michelle, Xia, Sunny, Li, Canhui, Wong, Amy P., Wellhauser, Leigh, Molinski, Steven V., Ip, Wan, Ouyang, Hong, Avolio, Julie, Forman-Kay, Julie D., Ratjen, Felix, Hirota, Jeremy A., Rommens, Johanna, Rossant, Janet, Gonska, Tanja, Moraes, Theo J., Bear, Christine E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5481189/
https://www.ncbi.nlm.nih.gov/pubmed/28649446
http://dx.doi.org/10.1038/s41525-017-0015-6