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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...
Autores principales: | 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. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7883678/ https://www.ncbi.nlm.nih.gov/pubmed/33597881 http://dx.doi.org/10.3389/fphar.2020.616834 |
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