ArticleInternational journal of molecular sciences2020
Electrophysiological Abnormalities in VLCAD Deficient hiPSC-Cardiomyocytes Can Be Improved by Lowering Accumulation of Fatty Acid Oxidation Intermediates.
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Article in International journal of molecular sciences, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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22citing papers in PubMed
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Who cites it
22 citing papers in PubMed.
- Metabolic cardiomyopathies: untangling clinical heterogeneity with human stem-cell derived models.EMBO molecular medicine · 2025Review
- The Pathogenesis of Very Long-Chain Acyl-CoA Dehydrogenase Deficiency.Biomolecules · 2025Review
- Metabolic Flexibility of the Heart: The Role of Fatty Acid Metabolism in Health, Heart Failure, and Cardiometabolic Diseases.International journal of molecular sciences · 2024Review
- Transcriptome analysis of effects ofOpen medicine (Warsaw, Poland) · 2024Article
- Article
- Maturation of hiPSC-derived cardiomyocytes promotes adult alternative splicing of SCN5A and reveals changes in sodium current associated with cardiac arrhythmia.Cardiovascular research · 2023Article
- Effects of High-Fat Diet on Cardiovascular Protein Expression in Mice Based on Proteomics.Diabetes, metabolic syndrome and obesity : targets and therapy · 2023Article
- Injection of IFrontiers in physiology · 2023Article
- Mitochondrial hepatopathy: Anticipated difficulties in management of fatty acid oxidation defects and urea cycle defects.World journal of hepatology · 2022Review
- Review
- hiPSC-Derived Cells as Models for Drug Discovery.International journal of molecular sciences · 2021Article
- Modelling Mitochondrial Disease in Human Pluripotent Stem Cells: What Have We Learned?International journal of molecular sciences · 2021Review
- Human-induced pluripotent stem cell-derived cardiomyocytes, 3D cardiac structures, and heart-on-a-chip as tools for drug research.Pflugers Archiv : European journal of physiology · 2021Review
- Successful orthotopic heart transplantation in CPTII deficiency.Molecular genetics and metabolism · 2021Article
- The Antibiotic Doxycycline Impairs Cardiac Mitochondrial and Contractile Function.International journal of molecular sciences · 2021Article
- Recent Advances in Modeling Mitochondrial Cardiomyopathy Using Human Induced Pluripotent Stem Cells.Frontiers in cell and developmental biology · 2021Review
- Cardioprotection stimulated by resveratrol and grape products prevents lethal cardiac arrhythmias in an animal model of ischemia and reperfusion.Acta cirurgica brasileira · 2021Article
- Diagnosis, genetic characterization and clinical follow up of mitochondrial fatty acid oxidation disorders in the new era of expanded newborn screening: A single centre experience.Molecular genetics and metabolism reports · 2020Article
- Mitochondrial Fatty Acid Oxidation Disorders: Laboratory Diagnosis, Pathogenesis, and the Complicated Route to Treatment.Journal of lipid and atherosclerosis · 2020Review
- Electrophysiological Abnormalities in VLCAD Deficient hiPSC-Cardiomyocytes Do not Improve with Carnitine Supplementation.Frontiers in pharmacology · 2020Article
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Authors and funding
19 authors.
Suzan J G KnottnerusLaboratory Genetic Metabolic Diseases, Amsterdam UMC, University of Amsterdam, Amsterdam Gastroenterology and Metabolism, Amsterdam Cardiovascular Sciences, 1105 AZ Amsterdam, The Netherlands.ORCID 0000-0003-4961-6968
Isabella MengarelliDepartment of Clinical and Experimental Cardiology, Heart Center, Amsterdam Cardiovascular Sciences, Amsterdam UMC, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.
Rob C I WüstLaboratory Genetic Metabolic Diseases, Amsterdam UMC, University of Amsterdam, Amsterdam Gastroenterology and Metabolism, Amsterdam Cardiovascular Sciences, 1105 AZ Amsterdam, The Netherlands.
Antonius BaartscheerDepartment of Clinical and Experimental Cardiology, Heart Center, Amsterdam Cardiovascular Sciences, Amsterdam UMC, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.
Jeannette C BleekerLaboratory Genetic Metabolic Diseases, Amsterdam UMC, University of Amsterdam, Amsterdam Gastroenterology and Metabolism, Amsterdam Cardiovascular Sciences, 1105 AZ Amsterdam, The Netherlands.ORCID 0000-0001-9092-575X
Ruben CoronelDepartment of Clinical and Experimental Cardiology, Heart Center, Amsterdam Cardiovascular Sciences, Amsterdam UMC, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.
Sacha FerdinandusseLaboratory Genetic Metabolic Diseases, Amsterdam UMC, University of Amsterdam, Amsterdam Gastroenterology and Metabolism, Amsterdam Cardiovascular Sciences, 1105 AZ Amsterdam, The Netherlands.
Kaomei GuanInstitute of Pharmacology and Toxicology, Technische Universität Dresden, 01069 Dresden, Germany.ORCID 0000-0002-0753-3083
Lodewijk IJlstLaboratory Genetic Metabolic Diseases, Amsterdam UMC, University of Amsterdam, Amsterdam Gastroenterology and Metabolism, Amsterdam Cardiovascular Sciences, 1105 AZ Amsterdam, The Netherlands.
Wener LiInstitute of Pharmacology and Toxicology, Technische Universität Dresden, 01069 Dresden, Germany.
Xiaojing LuoInstitute of Pharmacology and Toxicology, Technische Universität Dresden, 01069 Dresden, Germany.
Vincent M PorteroDepartment of Clinical and Experimental Cardiology, Heart Center, Amsterdam Cardiovascular Sciences, Amsterdam UMC, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.ORCID 0000-0002-7221-859X
Ying UlbrichtInstitute of Pharmacology and Toxicology, Technische Universität Dresden, 01069 Dresden, Germany.
Gepke VisserLaboratory Genetic Metabolic Diseases, Amsterdam UMC, University of Amsterdam, Amsterdam Gastroenterology and Metabolism, Amsterdam Cardiovascular Sciences, 1105 AZ Amsterdam, The Netherlands.
Ronald J A WandersLaboratory Genetic Metabolic Diseases, Amsterdam UMC, University of Amsterdam, Amsterdam Gastroenterology and Metabolism, Amsterdam Cardiovascular Sciences, 1105 AZ Amsterdam, The Netherlands.
Frits A WijburgDepartment of Paediatric Metabolic Diseases, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.
Arie O VerkerkDepartment of Clinical and Experimental Cardiology, Heart Center, Amsterdam Cardiovascular Sciences, Amsterdam UMC, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.ORCID 0000-0003-2140-834X
Riekelt H HoutkooperLaboratory Genetic Metabolic Diseases, Amsterdam UMC, University of Amsterdam, Amsterdam Gastroenterology and Metabolism, Amsterdam Cardiovascular Sciences, 1105 AZ Amsterdam, The Netherlands.ORCID 0000-0001-9961-0842
Connie R BezzinaDepartment of Clinical and Experimental Cardiology, Heart Center, Amsterdam Cardiovascular Sciences, Amsterdam UMC, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.
Funding
Fondation Leducq RHYTHM 16CVD 02Hartstichting CVON, PREDICT2Nederlandse Organisatie voor Wetenschappelijk Onderzoek VICI, 0.16.150.610Universiteit van Amsterdam Impulse grant 2016Velux Stiftung 1063ZonMw 91715305
Abstract
Patients with very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) can present with life-threatening cardiac arrhythmias. The pathophysiological mechanism is unknown. We reprogrammed fibroblasts from one mildly and one severely affected VLCADD patient, into human induced pluripotent stem cells (hiPSCs) and differentiated these into cardiomyocytes (VLCADD-CMs). VLCADD-CMs displayed shorter action potentials (APs), more delayed afterdepolarizations (DADs) and higher systolic and diastolic intracellular Ca
Indexed as
Action PotentialsAcyl-CoA Dehydrogenase, Long-ChainArrhythmias, CardiacCardiac ElectrophysiologyCongenital Bone Marrow Failure SyndromesEpoxy CompoundsFatty AcidsHumansInduced Pluripotent Stem CellsLipid Metabolism, Inborn ErrorsMitochondriaMitochondrial DiseasesMuscular DiseasesMyocytes, CardiacOxidation-ReductionResveratrolAcyl-CoA Dehydrogenase, Long-ChainEpoxy CompoundsetomoxirFatty AcidsResveratrolacylcarnitinesarrhythmiashiPSCVLCADD
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.