Electrophysiological Abnormalities in VLCAD Deficient hiPSC-Cardiomyocytes Do not Improve with Carnitine Supplementation

Patients with a deficiency in very long-chain acyl-CoA dehydrogenase (VLCAD), an enzyme that is involved in the mitochondrial beta-oxidation of long-chain fatty acids, are at risk for developing cardiac arrhythmias. In human induced pluripotent stem cell derived cardiomyocytes (hiPSC-CMs), VLCAD def...

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Enregistré dans:
Détails bibliographiques
Publié dans:Front Pharmacol
Auteurs principaux: Verkerk, Arie O., Knottnerus, Suzan J. G., Portero, Vincent, Bleeker, Jeannette C., Ferdinandusse, Sacha, Guan, Kaomei, IJlst, Lodewijk, Visser, Gepke, Wanders, Ronald J. A., Wijburg, Frits A., Bezzina, Connie R., Mengarelli, Isabella, Houtkooper, Riekelt H.
Format: Artigo
Langue:Inglês
Publié: Frontiers Media S.A. 2021
Sujets:
Pharmacology
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC7883678/
https://ncbi.nlm.nih.gov/pubmed/33597881
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3389/fphar.2020.616834
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