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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Gepubliceerd in:JCI Insight
Hoofdauteurs: 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.
Formaat: Artigo
Taal:Inglês
Gepubliceerd in: American Society for Clinical Investigation 2016
Onderwerpen:
Clinical Medicine
Online toegang: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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