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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Detalles Bibliográficos
Autores principales: 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.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7177397/
https://www.ncbi.nlm.nih.gov/pubmed/32276429
http://dx.doi.org/10.3390/ijms21072589

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7177397/
https://www.ncbi.nlm.nih.gov/pubmed/32276429
http://dx.doi.org/10.3390/ijms21072589

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