Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation
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Ivacaftor for patients with cystic fibrosisUpdate in Cystic Fibrosis 2014Potentiators (specific therapies for class III and IV mutations) for cystic fibrosisCurrent and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implicationsAnimal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and DivergencesNew and emerging targeted therapies for cystic fibrosisComputed tomography dose optimisation in cystic fibrosis: A reviewTargeted therapies to improve CFTR function in cystic fibrosisCystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectivesCFTR Modulators: Shedding Light on Precision Medicine for Cystic FibrosisCurrent and Emerging Therapies for the Treatment of Cystic Fibrosis or Mitigation of Its SymptomsA functional CFTR assay using primary cystic fibrosis intestinal organoidsHarnessing public domain data to discover and validate therapeutic targets.Clinical Pharmacogenetics Implementation Consortium (CPIC) guidelines for ivacaftor therapy in the context of CFTR genotypeIn vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N-of-1 studies.Cystic fibrosis transmembrane conductance regulator mutations at a referral center for cystic fibrosis.CFTR Modulators for the Treatment of Cystic Fibrosis.Cystic fibrosis transmembrane conductance regulator (CFTR) potentiators protect G551D but not ΔF508 CFTR from thermal instability.State of progress in treating cystic fibrosis respiratory disease.Lumacaftor and ivacaftor in the management of patients with cystic fibrosis: current evidence and future prospects.Apical CFTR expression in human nasal epithelium correlates with lung disease in cystic fibrosis.Ivacaftor: a novel mutation modulating drugPotentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis.PharmGKB summary: very important pharmacogene information for CFTRRecent advances in understanding and managing cystic fibrosis transmembrane conductance regulator dysfunction.Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.Improved fluorescence assays to measure the defects associated with F508del-CFTR allow identification of new active compounds.Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.Ivacaftor: a novel gene-based therapeutic approach for cystic fibrosisAcute administration of ivacaftor to people with cystic fibrosis and a G551D-CFTR mutation reveals smooth muscle abnormalitiesCystic fibrosis: a model system for precision medicine.Development, clinical utility, and place of ivacaftor in the treatment of cystic fibrosis.Progress in cystic fibrosis and the CF Therapeutics Development NetworkFunctional Rescue of F508del-CFTR Using Small Molecule CorrectorsAn update of childhood genetic disorders.From pipeline to patient: new developments in cystic fibrosis therapeutics.Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health.Repairing mutated proteins--development of small molecules targeting defects in the cystic fibrosis transmembrane conductance regulator.Managing the underlying cause of cystic fibrosis: a future role for potentiators and correctors.Cystic fibrosis transmembrane regulator correctors and potentiators.
P2860
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P2860
Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年学术文章
@wuu
2012年学术文章
@zh-cn
2012年学术文章
@zh-hans
2012年学术文章
@zh-my
2012年学术文章
@zh-sg
2012年學術文章
@yue
2012年學術文章
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2012年學術文章
@zh-hant
name
Ivacaftor in subjects with cys ...... for the F508del-CFTR mutation
@ast
Ivacaftor in subjects with cys ...... for the F508del-CFTR mutation
@en
type
label
Ivacaftor in subjects with cys ...... for the F508del-CFTR mutation
@ast
Ivacaftor in subjects with cys ...... for the F508del-CFTR mutation
@en
prefLabel
Ivacaftor in subjects with cys ...... for the F508del-CFTR mutation
@ast
Ivacaftor in subjects with cys ...... for the F508del-CFTR mutation
@en
P2093
P2860
P356
P1433
P1476
Ivacaftor in subjects with cys ...... for the F508del-CFTR mutation
@en
P2093
Claudia L Ordoñez
David E Geller
Drucy S Borowitz
Haihong Li
Patrick A Flume
VX 08-770-104 Study Group
P2860
P304
P356
10.1378/CHEST.11-2672
P407
P577
2012-09-01T00:00:00Z