Ivacaftor potentiation of multiple CFTR channels with gating mutations.
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Ivacaftor for patients with cystic fibrosisTargeted 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 FibrosisPotentiation of ΔF508- and G551D-CFTR-Mediated Cl- Current by Novel HydroxypyrazolinesThe cystic fibrosis transmembrane conductance regulator (CFTR) and its stabilityA little CFTR goes a long way: CFTR-dependent sweat secretion from G551D and R117H-5T cystic fibrosis subjects taking ivacaftorClinical Pharmacogenetics Implementation Consortium (CPIC) guidelines for ivacaftor therapy in the context of CFTR genotypeRelease of cystic fibrosis airway inflammatory markers from Pseudomonas aeruginosa-stimulated human neutrophils involves NADPH oxidase-dependent extracellular DNA trap formation.In vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N-of-1 studies.Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftorPhenotypic profiling of CFTR modulators in patient-derived respiratory epithelia.Lumacaftor/ivacaftor combination for cystic fibrosis patients homozygous for Phe508del-CFTR.Decoding F508del misfolding in cystic fibrosis.Three charged amino acids in extracellular loop 1 are involved in maintaining the outer pore architecture of CFTR.Towards personalized agriculture: what chemical genomics can bring to plant biotechnologyCystic fibrosis transmembrane conductance regulator (CFTR) potentiators protect G551D but not ΔF508 CFTR from thermal instability.A survey of detergents for the purification of stable, active human cystic fibrosis transmembrane conductance regulator (CFTR).Evolution of lung function during the first year of life in newborn screened cystic fibrosis infantsMeasurements of CFTR-mediated Cl- secretion in human rectal biopsies constitute a robust biomarker for Cystic Fibrosis diagnosis and prognosis.Lumacaftor and ivacaftor in the management of patients with cystic fibrosis: current evidence and future prospects.Modulation of CFTR gating by permeant ions.Discovery of novel potent ΔF508-CFTR correctors that target the nucleotide binding domain.Effect of genistein on basal jejunal chloride secretion in R117H CF mice is sex and route specific.PharmGKB summary: very important pharmacogene information for CFTRInteraction non grata between CFTR's correctors and potentiatorsSome gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.Ivacaftor in a young boy with the rare gating mutation S549R--use of lung clearance index to track progress: a case report.Normalization of sweat chloride concentration and clinical improvement with ivacaftor in a patient with cystic fibrosis with mutation S549N.Towards Increasing the Clinical Relevance of In Silico Methods to Predict Pathogenic Missense VariantsMurine and human CFTR exhibit different sensitivities to CFTR potentiators.Molecular modelling and molecular dynamics of CFTR.Functional defect of variants in the adenosine triphosphate-binding sites of ABCB4 and their rescue by the cystic fibrosis transmembrane conductance regulator potentiator, ivacaftor (VX-770).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.The magnitude of ivacaftor effects on fluid secretion via R117H-CFTR channels: Human in vivo measurementsCapturing the Direct Binding of CFTR Correctors to CFTR by Using Click Chemistry.Effect of ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation: patient-reported outcomes in the STRIVE randomized, controlled trialChannel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop.
P2860
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P2860
Ivacaftor potentiation of multiple CFTR channels with gating mutations.
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
2012年论文
@zh
2012年论文
@zh-cn
name
Ivacaftor potentiation of multiple CFTR channels with gating mutations.
@en
Ivacaftor potentiation of multiple CFTR channels with gating mutations.
@nl
type
label
Ivacaftor potentiation of multiple CFTR channels with gating mutations.
@en
Ivacaftor potentiation of multiple CFTR channels with gating mutations.
@nl
prefLabel
Ivacaftor potentiation of multiple CFTR channels with gating mutations.
@en
Ivacaftor potentiation of multiple CFTR channels with gating mutations.
@nl
P2093
P1476
Ivacaftor potentiation of multiple CFTR channels with gating mutations.
@en
P2093
Art Urrutia
Beth J Hoffman
Bill Burton
Chien-Jung Huang
Fredrick Van Goor
James P Johnson
Jennings Worley
John Joubran
P304
P356
10.1016/J.JCF.2011.12.005
P577
2012-01-30T00:00:00Z