Metabolic rescue ameliorates mitochondrial encephalo-cardiomyopathy in murine and human iPSC models of Leigh syndrome.

Jin-Young YoonNastaran DaneshgarYi ChuBiyi ChenMarco HeftiAjit VikramKaikobad IraniLong-Sheng SongCharles BrennerE Dale AbelBarry LondonDao-Fu Dai

Published in: Clinical and translational medicine (2022)

Our study reveals direct mechanistic explanations of the observed cardiac bradyarrhythmia, diastolic dysfunction and neuronal apoptosis in mouse and human induced pluripotent stem cells (iPSC) models of LS.

Keyphrases

induced pluripotent stem cells
oxidative stress
left ventricular
endothelial cells
blood pressure
cell death
mouse model
atrial fibrillation
pluripotent stem cells