Clinical effects of the three CFTR potentiator treatments curcumin, genistein and ivacaftor in patients with the CFTR-S1251N gating mutation.

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第一作者： Gitte,Berkers

第一单位： Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands.

作者： Gitte,Berkers ^[1] ; Renske,van der Meer ^[2] ; Peter,van Mourik ^[1] ; Annelotte M,Vonk ^[3] ; Evelien,Kruisselbrink ^[3] ; Sylvia Wf,Suen ^[3] ; Harry Gm,Heijerman ^[4] ; Christof J,Majoor ^[5] ; Gerard H,Koppelman ^[6] ; Jolt,Roukema ^[7] ; Hettie M,Janssens ^[8] ; Yolanda B,de Rijke ^[9] ; E Marleen,Kemper ^[10] ; Jeffrey M,Beekman ^[3] ; Cornelis K,van der Ent ^[11] ; Hugo R,de Jonge ^[12]

作者单位： Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands. ^[1] Department of Pulmonology, Haga Teaching Hospital, The Hague, the Netherlands. ^[2] Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands; Regenerative Medicine Center Utrecht, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands. ^[3] Department of Pulmonology, University Medical Center Utrecht, Utrecht, the Netherlands. ^[4] Department of Respiratory Medicine, Amsterdam University Medical Centers, Amsterdam, the Netherlands. ^[5] Department of Pediatric Pulmonology and Pediatric Allergology and GRIAC Research Institute, University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Groningen, the Netherlands. ^[6] Department of Pediatric Pulmonology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, the Netherlands. ^[7] Department of Pediatrics, division of Respiratory Medicine and Allergology, Erasmus Medical Center/Sophia Children's Hospital, University Hospital Rotterdam, the Netherlands. ^[8] Department of Clinical Chemistry, Erasmus Medical Center, University Hospital Rotterdam, the Netherlands. ^[9] Department of Pharmacy, Amsterdam University Medical Centers, Amsterdam, the Netherlands. ^[10] Department of Pediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Utrecht, the Netherlands. Electronic address: K.vanderent@umcutrecht.nl. ^[11] Department of Gastroenterology and Hepatology, Erasmus Medical Center, University Hospital Rotterdam, the Netherlands. ^[12]

关键词 CFTR Curcumin Cystic fibrosis Genistein Ivacaftor Organoids