Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function.
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Update in Cystic Fibrosis 2014Regulatory Crosstalk by Protein Kinases on CFTR Trafficking and Activity.Targeted therapies to improve CFTR function in cystic fibrosisEpidemiology and genetics of cystic fibrosis in Asia: In preparation for the next-generation treatmentsCFTR Modulators: Shedding Light on Precision Medicine for Cystic FibrosisLMTK2-mediated phosphorylation regulates CFTR endocytosis in human airway epithelial cells.In vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N-of-1 studies.Lumacaftor/ivacaftor combination for cystic fibrosis patients homozygous for Phe508del-CFTR.Constrained bithiazoles: small molecule correctors of defective ΔF508-CFTR protein trafficking.Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis.Modulation of CFTR gating by permeant ions.PharmGKB summary: very important pharmacogene information for CFTRNovel personalized therapies for cystic fibrosis: treating the basic defect in all patients.Measurements of Functional Responses in Human Primary Lung Cells as a Basis for Personalized Therapy for Cystic Fibrosis.Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.Benign outcome among positive cystic fibrosis newborn screen children with non-CF-causing variants.Murine and human CFTR exhibit different sensitivities to CFTR potentiators.Improved fluorescence assays to measure the defects associated with F508del-CFTR allow identification of new active compounds.Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: a double-blind, randomised controlled trial.Hallmarks of therapeutic management of the cystic fibrosis functional landscape.Deletion of Phenylalanine 508 in the First Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator Increases Conformational Exchange and Inhibits Dimerization.The magnitude of ivacaftor effects on fluid secretion via R117H-CFTR channels: Human in vivo measurementsChannel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop.From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations.Positioning of extracellular loop 1 affects pore gating of the cystic fibrosis transmembrane conductance regulatorImproving newborn screening for cystic fibrosis using next-generation sequencing technology: a technical feasibility studyCreation and characterization of an airway epithelial cell line for stable expression of CFTR variants.Therapeutic Approaches to Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis.Analysis of cystic fibrosis-associated P67L CFTR illustrates barriers to personalized therapeutics for orphan diseasesCFTR: A New Horizon in the Pathomechanism and Treatment of Pancreatitis.Correctors and Potentiators Rescue Function of the Truncated W1282X-Cystic Fibrosis Transmembrane Regulator (CFTR) Translation ProductEmerging drugs for cystic fibrosis.Cystic fibrosis genetics: from molecular understanding to clinical application.Breakthrough therapies: Cystic fibrosis (CF) potentiators and correctors.2014 year in review: Cystic fibrosis.Repairing the basic defect in cystic fibrosis - one approach is not enough.Searching for a cure for cystic fibrosis. A 25-year quest in a nutshell.Stabilization of a nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator yields insight into disease-causing mutations.Thymosin α1 represents a potential potent single-molecule-based therapy for cystic fibrosisTwo Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation.
P2860
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P2860
Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function.
description
2013 nî lūn-bûn
@nan
2013年の論文
@ja
2013年論文
@yue
2013年論文
@zh-hant
2013年論文
@zh-hk
2013年論文
@zh-mo
2013年論文
@zh-tw
2013年论文
@wuu
2013年论文
@zh
2013年论文
@zh-cn
name
Effect of ivacaftor on CFTR fo ...... rotein processing or function.
@en
Effect of ivacaftor on CFTR fo ...... rotein processing or function.
@nl
type
label
Effect of ivacaftor on CFTR fo ...... rotein processing or function.
@en
Effect of ivacaftor on CFTR fo ...... rotein processing or function.
@nl
prefLabel
Effect of ivacaftor on CFTR fo ...... rotein processing or function.
@en
Effect of ivacaftor on CFTR fo ...... rotein processing or function.
@nl
P2093
P1476
Effect of ivacaftor on CFTR fo ...... rotein processing or function.
@en
P2093
Beth J Hoffman
Bill Burton
Fredrick Van Goor
P356
10.1016/J.JCF.2013.06.008
P577
2013-07-23T00:00:00Z