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ivacaftor

Known as: 3-quinolinecarboxamide, N-(2,4-bis(1,1-dimethylethyl)-5-hydroxyphenyl)-1,4-dihydro-4-oxo-, N-(2,4-bis(1,1-dimethylethyl)-5-hydroxyphenyl)-4-oxo-1,4-dihydroquinoline-3-carboxamide, N-(2,4-di-tert-butyl-5-hydroxyphenyl)-4-oxo-1,4-dihydroquinoline-3-carboxamide 
National Institutes of Health

Related topics

Related topics

10 relations

Broader (3)

AminophenolsChloride Channel AgonistsQuinolones
Chloride Channel Activation Potentiators [MoA]ivacaftor / lumacaftorivacaftor 125 MG / lumacaftor 200 MG Oral Tablet [ORKAMBI]ivacaftor 150 MG Oral Tablet [Kalydeco]

Narrower (3)

KalydecoORKAMBIVX-770

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
2020
2020
Clinical Effectiveness of Lumacaftor/Ivacaftor in Cystic Fibrosis Patients Homozygous for F508del-CFTR.
RATIONALE The combination of lumacaftor and ivacaftor (LUM/IVA) is an approved cystic fibrosis transmembrane conductance… 
2018
2018
Potentiation of the cystic fibrosis transmembrane conductance regulator Cl- channel by ivacaftor is temperature independent.
Ivacaftor is the first drug to target directly defects in the cystic fibrosis transmembrane conductance regulator (CFTR), which… 
2015
2015
Effect of ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation: patient-reported outcomes in the STRIVE randomized, controlled trial
BackgroundCystic fibrosis (CF) is an inherited, rare autosomal recessive disease that results in chronically debilitating… 
2015
2015
Concentration of fractional excretion of nitric oxide (FENO): A potential airway biomarker of restored CFTR function.
2015
2015
The Role of Ivacaftor in Severe Cystic Fibrosis in a Patient With the R117H Mutation.
Cystic fibrosis (CF) conductance transmembrane regulator functions as a chloride (Cl-) channel in multiple organs, including the… 
Review
2014
Review
2014
Personalised medicine in cystic fibrosis is unaffordable.
2014
2014
Improvement in Bronchiectasis on CT Imaging in a Pediatric Patient with Cystic Fibrosis on Ivacaftor Therapy
sis 11 years earlier. Her body mass index increased from 16.2 to 21.2 after 2 years of therapy, while her spirometry showed… 
2014
2014
False dawn for cystic fibrosis disease modifiers?
Recent results have cast doubt over how effective a breakthrough class of cystic fibrosis drugs can be in patients with the most… 
Review
2013
Review
2013
Ivacaftor: the first therapy acting on the primary cause of cystic fibrosis.
Cystic fibrosis (CF) is a life-shortening disorder that affects over 30,000 people in the U.S. and 70,000 worldwide. CF is caused… 
Review
2012
Review
2012
Cystic Fibrosis Transmembrane Conductance Regulator–Modifying Medications: The Future of Cystic Fibrosis Treatment
OBJECTIVE: To review and evaluate cystic fibrosis transmembrane conductance regulator (CFTR) modulators for the treatment of… 
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