From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations.
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Epithelial Anion Transport as Modulator of Chemokine SignalingCFTR Modulators: Shedding Light on Precision Medicine for Cystic FibrosisRibosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression DefectHuman pluripotent stem cell-derived acinar/ductal organoids generate human pancreas upon orthotopic transplantation and allow disease modelling.Alteration of protein function by a silent polymorphism linked to tRNA abundance.ABCMdb reloaded: updates on mutations in ATP binding cassette proteins.Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia.Molecular modelling and molecular dynamics of CFTR.Correctors and Potentiators Rescue Function of the Truncated W1282X-Cystic Fibrosis Transmembrane Regulator (CFTR) Translation ProductPharmacological Rescue of Conditionally Reprogrammed Cystic Fibrosis Bronchial Epithelial Cells.Restoration of R117H CFTR folding and function in human airway cells through combination treatment with VX-809 and VX-770Specific stabilization of CFTR by phosphatidylserine.Efficacy of lumacaftor-ivacaftor for the treatment of cystic fibrosis patients homozygous for the F508del-CFTR mutation.Individualized medicine using intestinal responses to CFTR potentiators and correctors.CFTR pharmacology.Regulation of mRNA turnover in cystic fibrosis lung disease.Gene delivery to the lungs: pulmonary gene therapy for cystic fibrosis.Animal models of biliary injury and altered bile acid metabolism.Drug-induced dyspnea versus cystic fibrosis exacerbation: a diagnostic dilemma.Stem cell-derived organoids to model gastrointestinal facets of cystic fibrosis.Cas9/gRNA targeted excision of cystic fibrosis-causing deep-intronic splicing mutations restores normal splicing of CFTR mRNA.CFTR-NHERF2-LPA₂ Complex in the Airway and Gut Epithelia.Attenuation of Phosphorylation-dependent Activation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by Disease-causing Mutations at the Transmission Interface.Strategies for the etiological therapy of cystic fibrosis.Highlights from the 2015 North American Cystic Fibrosis Conference.Partial rescue of F508del-CFTR channel gating with modest improvement of protein processing, but not stability by a dual-acting small molecule.AJRCCM: 100-Year Anniversary. Progress along the Pathway of Discovery Leading to Treatment and Cure of Cystic Fibrosis.Mutation-specific downregulation of CFTR2 variants by gating potentiators.Genetic Complexity of Autosomal Dominant Polycystic Kidney and Liver Diseases.Germline genetic variants with implications for disease risk and therapeutic outcomes.A common mechanism for CFTR potentiators.Personalized Medicine in CF: From Modulator Development to Therapy for Cystic Fibrosis Patients with Rare CFTR Mutations.Extracellular oxidation in Cystic Fibrosis airway epithelium causes enhanced EGFR/ADAM17 activity.Pharmacoperones as Novel Therapeutics for Diverse Protein Conformational Diseases.Transformative therapies for rare CFTR missense alleles.Perforin proteostasis is regulated through its C2 domain: supra-physiological cell death mediated by T431D-perforin.Molecular Mechanism of Action of Trimethylangelicin Derivatives as CFTR Modulators.Ion Channel Modulators in Cystic FibrosisPeripheral Protein Quality Control as a Novel Drug Target for CFTR StabilizerEmerging microRNA Therapeutic Approaches for Cystic Fibrosis
P2860
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P2860
From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations.
description
2016 nî lūn-bûn
@nan
2016年の論文
@ja
2016年論文
@yue
2016年論文
@zh-hant
2016年論文
@zh-hk
2016年論文
@zh-mo
2016年論文
@zh-tw
2016年论文
@wuu
2016年论文
@zh
2016年论文
@zh-cn
name
From CFTR biology toward combi ...... of cystic fibrosis mutations.
@ast
From CFTR biology toward combi ...... of cystic fibrosis mutations.
@en
type
label
From CFTR biology toward combi ...... of cystic fibrosis mutations.
@ast
From CFTR biology toward combi ...... of cystic fibrosis mutations.
@en
prefLabel
From CFTR biology toward combi ...... of cystic fibrosis mutations.
@ast
From CFTR biology toward combi ...... of cystic fibrosis mutations.
@en
P2093
P2860
P356
P1476
From CFTR biology toward combi ...... of cystic fibrosis mutations.
@en
P2093
Annette N Chiang
David N Sheppard
Douglas M Cyr
Eric J Sorscher
Garry R Cutting
Harvey B Pollard
Jeffrey L Brodsky
Jeong S Hong
Kathryn W Peters
Radu G Avramescu
P2860
P304
P356
10.1091/MBC.E14-04-0935
P577
2016-02-01T00:00:00Z