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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Vydáno v:Int J Mol Sci
Hlavní autoři: 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.
Médium: Artigo
Jazyk:Inglês
Vydáno: MDPI 2020
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On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC7177397/
https://ncbi.nlm.nih.gov/pubmed/32276429
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3390/ijms21072589
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