Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809. - Wikidata - awgldk - TriplyDB

Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.

Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.

http://www.wikidata.org/entity/Q35558817

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Update in Cystic Fibrosis 2014 Correctors (specific therapies for class II CFTR mutations) for cystic fibrosis New and emerging targeted therapies for cystic fibrosis Human induced pluripotent stem cells for monogenic disease modelling and therapy Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis Regulatory Crosstalk by Protein Kinases on CFTR Trafficking and Activity.Targeted therapies to improve CFTR function in cystic fibrosis Cystic fibrosis transmembrane conductance regulator dysfunction and its treatment Pharmacological chaperoning: a primer on mechanism and pharmacology Emerging novel concept of chaperone therapies for protein misfolding diseases Cystic fibrosis therapeutics: the road ahead Early pulmonary inflammation and lung damage in children with cystic fibrosis Modulation of the maladaptive stress response to manage diseases of protein folding A physiologically-motivated compartment-based model of the effect of inhaled hypertonic saline on mucociliary clearance and liquid transport in cystic fibrosis Requirements for Efficient Correction of ΔF508 CFTR Revealed by Analyses of Evolved Sequences Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectives CFTR Modulators: Shedding Light on Precision Medicine for Cystic Fibrosis Current and Emerging Therapies for the Treatment of Cystic Fibrosis or Mitigation of Its Symptoms Airway hydration and COPD A functional CFTR assay using primary cystic fibrosis intestinal organoids Functional rescue of a kidney anion exchanger 1 trafficking mutant in renal epithelial cells Allosteric coupling between the intracellular coupling helix 4 and regulatory sites of the first nucleotide-binding domain of CFTR Rescue of NBD2 mutants N1303K and S1235R of CFTR by small-molecule correctors and transcomplementation Pseudomonas aeruginosa Reduces VX-809 Stimulated F508del-CFTR Chloride Secretion by Airway Epithelial Cells Potentiation of ΔF508- and G551D-CFTR-Mediated Cl- Current by Novel Hydroxypyrazolines Ribosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression Defect Mechanistic Approaches to Improve Correction of the Most Common Disease-Causing Mutation in Cystic Fibrosis Increasing the Endoplasmic Reticulum Pool of the F508del Allele of the Cystic Fibrosis Transmembrane Conductance Regulator Leads to Greater Folding Correction by Small Molecule Therapeutics Cystic fibrosis related liver disease--another black box in hepatology.Curcumin and genistein: the combined effects on disease-associated CFTR mutants and their clinical implications Barriers to inhaled gene therapy of obstructive lung diseases: A review Malfolded protein structure and proteostasis in lung diseases.The cystic fibrosis transmembrane conductance regulator (CFTR) and its stability LMTK2-mediated phosphorylation regulates CFTR endocytosis in human airway epithelial cells.Small-molecule structure correctors target abnormal protein structure and function: structure corrector rescue of apolipoprotein E4-associated neuropathology The ΔF508-CFTR mutation inhibits wild-type CFTR processing and function when co-expressed in human airway epithelia and in mouse nasal mucosa.Enhancing the Potency of F508del Correction: A Multi-Layer Combinational Approach to Drug Discovery for Cystic Fibrosis.Clinical Pharmacogenetics Implementation Consortium (CPIC) guidelines for ivacaftor therapy in the context of CFTR genotype Synergy-based small-molecule screen using a human lung epithelial cell line yields ΔF508-CFTR correctors that augment VX-809 maximal efficacy.In vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N-of-1 studies.

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Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.

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Proceedings of the National Academy of Sciences of the United States of America

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Correction of the F508del-CFTR ...... e investigational drug VX-809.

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Bill Burton

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Caroline J Decker

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Eric R Olson

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Fredrick Van Goor

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Jason McCartney

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Jeffrey H Stack

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Jeffrey J Wine

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Kimberly S Straley

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Mark Miller

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Melissa Ashlock

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P2860

Cystic Fibrosis: Lessons from the Sweat Gland

Chloride channels as drug targets.

Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis

Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report

Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA

Cystic fibrosis: a worldwide analysis of CFTR mutations--correlation with incidence data and application to screening

Probing protein structure by limited proteolysis.

Additive effect of multiple pharmacological chaperones on maturation of CFTR processing mutants

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