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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Pubblicato in:Int J Mol Sci
Autori principali: 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.
Natura: Artigo
Lingua:Inglês
Pubblicazione: MDPI 2020
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Accesso online: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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