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

• Main Authors: Arie O. Verkerk (Author), Suzan J. G. Knottnerus (Author), Vincent Portero (Author), Jeannette C. Bleeker (Author), Sacha Ferdinandusse (Author), Kaomei Guan (Author), Lodewijk IJlst (Author), Gepke Visser (Author), Ronald J. A. Wanders (Author), Frits A. Wijburg (Author), Connie R. Bezzina (Author), Isabella Mengarelli (Author), Riekelt H. Houtkooper (Author)
• Format: Book
• Published: Frontiers Media S.A., 2021-01-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

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...