Cargando…
Anti-Infectives Restore ORKAMBI(®) Rescue of F508del-CFTR Function in Human Bronchial Epithelial Cells Infected with Clinical Strains of P. aeruginosa
Chronic infection and inflammation are the primary causes of declining lung function in Cystic Fibrosis (CF) patients. ORKAMBI(®) (Lumacaftor-Ivacaftor) is an approved combination therapy for Cystic Fibrosis (CF) patients bearing the most common mutation, F508del, in the cystic fibrosis conductance...
Autores principales: | Laselva, Onofrio, Stone, Tracy A., Bear, Christine E., Deber, Charles M. |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2020
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7072183/ https://www.ncbi.nlm.nih.gov/pubmed/32092967 http://dx.doi.org/10.3390/biom10020334 |
Ejemplares similares
-
Treatment of Cystic Fibrosis Patients Homozygous for F508del with Lumacaftor-Ivacaftor (Orkambi(®)) Restores Defective CFTR Channel Function in Circulating Mononuclear Cells
por: Favia, Maria, et al.
Publicado: (2020) -
SFPQ rescues F508del-CFTR expression and function in cystic fibrosis bronchial epithelial cells
por: Kumar, Parameet, et al.
Publicado: (2021) -
Functional Rescue of F508del-CFTR Using Small Molecule Correctors
por: Molinski, Steven, et al.
Publicado: (2012) -
Proximity Profiling of the CFTR Interaction Landscape in Response to Orkambi
por: Iazzi, Melissa, et al.
Publicado: (2022) -
Tgf-β1 Inhibits Cftr Biogenesis and Prevents Functional Rescue of ΔF508-Cftr in Primary Differentiated Human Bronchial Epithelial Cells
por: Snodgrass, Steven M., et al.
Publicado: (2013)