Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment.
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Correctors (specific therapies for class II CFTR mutations) for cystic fibrosisUltra-rare Disease and Genomics-Driven Precision MedicineSteviol reduces MDCK Cyst formation and growth by inhibiting CFTR channel activity and promoting proteasome-mediated CFTR degradationNT5E mutations that cause human disease are associated with intracellular mistrafficking of NT5E proteinLMTK2-mediated phosphorylation regulates CFTR endocytosis in human airway epithelial cells.Reduced blood pressure of CFTR-F508del carriers correlates with diminished arterial reactivity rather than circulating blood volume in miceClinical Pharmacogenetics Implementation Consortium (CPIC) guidelines for ivacaftor therapy in the context of CFTR genotypeCFTR and TNR-CFTR expression and function in the kidney.Global and disease-associated genetic variation in the human Fanconi anemia gene family.F508del-cystic fibrosis transmembrane regulator correctors for treatment of cystic fibrosis: a patent review.Inhibiting an Epoxide Hydrolase Virulence Factor from Pseudomonas aeruginosa Protects CFTR.Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.A Genotypic-Oriented View of CFTR Genetics Highlights Specific Mutational Patterns Underlying Clinical Macrocategories of Cystic FibrosisLow temperature and chemical rescue affect molecular proximity of DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) and epithelial sodium channel (ENaC)Suppression of premature termination codons as a therapeutic approach.c-Cbl reduces stability of rescued ∆F508-CFTR in human airway epithelial cells: Implications for cystic fibrosis treatment.Lentiviral-mediated phenotypic correction of cystic fibrosis pigs.Cystic fibrosis - a multiorgan protein misfolding disease.R248G cystic fibrosis transmembrane conductance regulator mutation in three siblings presenting with recurrent acute pancreatitis and reproductive issues: a case series.Lactoferrin: an alternative view of its role in human biological fluids.Molecular motors and apical CFTR traffic in epithelia.Ivacaftor treatment of cystic fibrosis patients with the G551D mutation: a review of the evidence.Ivacaftor: a review of its use in patients with cystic fibrosis.Nebulized liposomal amikacin for the treatment of Pseudomonas aeruginosa infection in cystic fibrosis patients.Progress towards next-generation therapeutics for cystic fibrosis.Efficacy and adverse effects of drugs used to treat adult cystic fibrosis.Management of Pseudomonas aeruginosa infection in cystic fibrosis patients using inhaled antibiotics with a focus on nebulized liposomal amikacinGenetic medicines for CF: Hype versus reality.Pharmacogenetics of cystic fibrosis treatment.Cystic Fibrosis and the Nervous System.A new insight into CFTR allele frequency in Brazil through next generation sequencing.Pseudohalide anions reveal a novel extracellular site for potentiators to increase CFTR function.Functional Gene Correction for Cystic Fibrosis in Lung Epithelial Cells Generated from Patient iPSCs.Defective CFTR causes cystic fibrosisInfluenza A matrix protein M2 downregulates CFTR: inhibition of chloride transport by a proton channel of the viral envelope.PharmGKB summary: ivacaftor pathway, pharmacokinetics/pharmacodynamics.Speeding Up the Identification of Cystic Fibrosis Transmembrane Conductance Regulator-Targeted Drugs: An Approach Based on Bioinformatics Strategies and Surface Plasmon Resonance.Defective CFTR does not transport Cl- from cytosol to extracellular regionDetection of CFTR protein in human leukocytes by flow cytometry.
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Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment.
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on June 2011
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
Cystic fibrosis transmembrane ...... r mutation-specific treatment.
@en
Cystic fibrosis transmembrane ...... r mutation-specific treatment.
@nl
type
label
Cystic fibrosis transmembrane ...... r mutation-specific treatment.
@en
Cystic fibrosis transmembrane ...... r mutation-specific treatment.
@nl
prefLabel
Cystic fibrosis transmembrane ...... r mutation-specific treatment.
@en
Cystic fibrosis transmembrane ...... r mutation-specific treatment.
@nl
P2093
P356
P1433
P1476
Cystic fibrosis transmembrane ...... r mutation-specific treatment.
@en
P2093
David A Stoltz
Douglas B Hornick
Mark P Rogan
P304
P356
10.1378/CHEST.10-2077
P407
P577
2011-06-01T00:00:00Z