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

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...

Full description

Bibliographic Details
Main Authors: Arie O. Verkerk, Suzan J. G. Knottnerus, Vincent Portero, Jeannette C. Bleeker, Sacha Ferdinandusse, Kaomei Guan, Lodewijk IJlst, Gepke Visser, Ronald J. A. Wanders, Frits A. Wijburg, Connie R. Bezzina, Isabella Mengarelli, Riekelt H. Houtkooper
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-01-01
Series:Frontiers in Pharmacology
Subjects:
very long-chain acyl-CoA dehydrogenase
arrhythmia < cardiovascular
acylcarnitines
action potential
human induced pluripotent stem cell derived cardiomyocytes
patients
Online Access:https://www.frontiersin.org/articles/10.3389/fphar.2020.616834/full

Internet

https://www.frontiersin.org/articles/10.3389/fphar.2020.616834/full

Similar Items