Acute administration of ivacaftor to people with cystic fibrosis and a G551D-CFTR mutation reveals smooth muscle abnormalities

BACKGROUND. Airflow obstruction is common in cystic fibrosis (CF), yet the underlying pathogenesis remains incompletely understood. People with CF often exhibit airway hyperresponsiveness, CF transmembrane conductance regulator (CFTR) is present in airway smooth muscle (ASM), and ASM from newborn CF...

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Detalhes bibliográficos
Publicado no:JCI Insight
Main Authors: Adam, Ryan J., Hisert, Katherine B., Dodd, Jonathan D., Grogan, Brenda, Launspach, Janice L., Barnes, Janel K., Gallagher, Charles G., Sieren, Jered P., Gross, Thomas J., Fischer, Anthony J., Cavanaugh, Joseph E., Hoffman, Eric A., Singh, Pradeep K., Welsh, Michael J., McKone, Edward F., Stoltz, David A.
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Clinical Investigation 2016
Assuntos:
Clinical Medicine
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4855508/
https://ncbi.nlm.nih.gov/pubmed/27158673
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/jci.insight.86183
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