Electrophysiological Abnormalities in VLCAD Deficient hiPSC-Cardiomyocytes Can Be Improved by Lowering Accumulation of Fatty Acid Oxidation Intermediates.

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作者： Suzan J G,Knottnerus ^[1] ; Isabella,Mengarelli ^[2] ; Rob C I,Wüst ^[3] ; Antonius,Baartscheer ^[1] ; Jeannette C,Bleeker ^[3] ; Ruben,Coronel ^[1] ; Sacha,Ferdinandusse ^[2] ; Kaomei,Guan ^[3] ; Lodewijk,IJlst ^[1] ; Wener,Li ^[4] ; Xiaojing,Luo ^[1] ; Vincent M,Portero ^[4] ; Ying,Ulbricht ^[4] ; Gepke,Visser ^[3] ; Ronald J A,Wanders ^[4] ; Frits A,Wijburg ^[1] ; Arie O,Verkerk ^[2] ; Riekelt H,Houtkooper ^[1] ; Connie R,Bezzina ^[5]

作者单位： Laboratory Genetic Metabolic Diseases, Amsterdam UMC, University of Amsterdam, Amsterdam Gastroenterology and Metabolism, Amsterdam Cardiovascular Sciences, 1105 AZ Amsterdam, The Netherlands. ^[1] Department of Paediatric Metabolic Diseases, Wilhelmina Children's Hospital, University Medical Center Utrecht, 3584 EA Utrecht, The Netherlands. ^[2] Department of Clinical and Experimental Cardiology, Heart Center, Amsterdam Cardiovascular Sciences, Amsterdam UMC, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands. ^[3] Institute of Pharmacology and Toxicology, Technische Universität Dresden, 01069 Dresden, Germany. ^[4] Department of Paediatric Metabolic Diseases, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands. ^[5] Department of Medical Biology, Amsterdam Cardiovascular Sciences, Amsterdam UMC, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands. ^[6]

关键词 VLCADD acylcarnitines arrhythmias hiPSC