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

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...

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Veröffentlicht in:Int J Mol Sci
Hauptverfasser: Knottnerus, Suzan J. G., Mengarelli, Isabella, Wüst, Rob C. I., Baartscheer, Antonius, Bleeker, Jeannette C., Coronel, Ruben, Ferdinandusse, Sacha, Guan, Kaomei, IJlst, Lodewijk, Li, Wener, Luo, Xiaojing, Portero, Vincent M., Ulbricht, Ying, Visser, Gepke, Wanders, Ronald J. A., Wijburg, Frits A., Verkerk, Arie O., Houtkooper, Riekelt H., Bezzina, Connie R.
Format: Artigo
Sprache:Inglês
Veröffentlicht: MDPI 2020
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Article
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC7177397/
https://ncbi.nlm.nih.gov/pubmed/32276429
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3390/ijms21072589
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