In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.
RC Rubenstein, ME Egan… - The Journal of clinical …, 1997 - Am Soc Clin Investig
… While the identity of the second mutant CFTR allele in these primary nasal epithelial cells
is unknown, we do know that it is not W1282X, F508, N1303K, G542X, G551D, or R553X. …
is unknown, we do know that it is not W1282X, F508, N1303K, G542X, G551D, or R553X. …
Functional activation of the cystic fibrosis trafficking mutant delta F508-CFTR by overexpression
… normal human airway epithelial cells also fail to detect CFTR, due to its low amounts. …
epithelial cells. Because we were unable to measure the low amount of CFTR protein in these cells…
epithelial cells. Because we were unable to measure the low amount of CFTR protein in these cells…
c-Cbl reduces stability of rescued∆ F508-CFTR in human airway epithelial cells: Implications for cystic fibrosis treatment
KM Cihil, A Zimnik… - … & integrative biology, 2013 - Taylor & Francis
… of ∆F508-CFTR may represent a … CFTR endocytosis, we propose that interfering with the
c-Cbl mediated endocytic recruitment of ∆F508-CFTR may increase stability of ∆508-CFTR in …
c-Cbl mediated endocytic recruitment of ∆F508-CFTR may increase stability of ∆508-CFTR in …
Functional stability of rescued ΔF508 cystic fibrosis transmembrane conductance regulator in airway epithelial cells
A Jurkuvenaite, L Chen, R Bartoszewski… - … of respiratory cell and …, 2010 - atsjournals.org
… CFTR in Human Airway Epithelial Cells In order to follow the trafficking of rescued ΔF508
CFTR at the cell … -4a on ΔF508 CFTR biogenesis in human airway epithelial cells (CFBE41o-ΔF…
CFTR at the cell … -4a on ΔF508 CFTR biogenesis in human airway epithelial cells (CFBE41o-ΔF…
… of delta F508 cystic fibrosis transmembrane conductance regulator protein and related chloride transport properties in the gallbladder epithelium from cystic fibrosis …
N Dray-Charier, A Paul, JY Scoazec, D Veissière… - Hepatology, 1999 - journals.lww.com
… expression, and chloride transport properties in gallbladder epithelial cells from nine F508
homozygous … Our results suggest that epithelial structural alterations aggravate F508 CFTR …
homozygous … Our results suggest that epithelial structural alterations aggravate F508 CFTR …
Rescue of ΔF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules
F Van Goor, KS Straley, D Cao… - … -Lung Cellular and …, 2006 - journals.physiology.org
… then tested in the FRT epithelial cell line expressing ΔF508-CFTR using Ussing chamber …
delta F508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR …
delta F508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR …
∆ F508 CFTR interactome remodelling promotes rescue of cystic fibrosis
S Pankow, C Bamberger, D Calzolari… - Nature, 2015 - nature.com
… The mutated CFTR anion channel is not fully … epithelial cells of patients with cystic fibrosis.
Low temperature or inhibition of histone deacetylases can partly rescue ∆F508 CFTR cellular …
Low temperature or inhibition of histone deacetylases can partly rescue ∆F508 CFTR cellular …
[HTML][HTML] Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells
… This analysis revealed that epithelial cells account for approximately 87% of all cells in the
non–CF, carrier, and CF samples, with no significant differences among the three groups (Fig. …
non–CF, carrier, and CF samples, with no significant differences among the three groups (Fig. …
Synergy-based small-molecule screen using a human lung epithelial cell line yields ΔF508-CFTR correctors that augment VX-809 maximal efficacy
PW Phuan, G Veit, J Tan, A Roldan, WE Finkbeiner… - Molecular …, 2014 - ASPET
… cystic fibrosis transmembrane conductance regulator (CFTR) mutation causing cystic fibrosis,
… primary cultures of human bronchial epithelial cells from a homozygous ∆F508 CF patient. (…
… primary cultures of human bronchial epithelial cells from a homozygous ∆F508 CF patient. (…
Trimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells
M Favia, MT Mancini, V Bezzerri… - … -Lung Cellular and …, 2014 - journals.physiology.org
… of human bronchial epithelial cells derived either from F508del CFTR homozygous donors,
CF-… delta F508 cystic fibrosis transmembrane conductance regulator in airway epithelial cells. …
CF-… delta F508 cystic fibrosis transmembrane conductance regulator in airway epithelial cells. …
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