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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Bibliografiset tiedot
Julkaisussa:JCI Insight
Päätekijät: 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.
Aineistotyyppi: Artigo
Kieli:Inglês
Julkaistu: American Society for Clinical Investigation 2016
Aiheet:
Clinical Medicine
Linkit: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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