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Stem cell function - Crossref

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Last Updated: 27 January 2022

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SNTA1 GeneRescues Ion Channel Function in Cardiomyocytes Derived from Induced Pluripotent Stem Cells Reprogrammed from Muscular Dystrophy Patients with Arrhythmias

Patients with Duchenne Muscular Dystrophy cardiomyopathy are at risk of obtaining life-threatening arrhythmias, but the mechanisms are unclear. In the development of arrhythmias in DMD patients, we investigated the role of cardiac ion channels controlling cardiac excitability. Two patients had abnormal ECGs with frequent runs of ventricular tachycardia. Protein levels were reduced in hemizygous DMD iPSC-CMs but not in heterozygous iPSC-CMs. In hemizygous iPSC-CMs restored channelosome function, I Na and I K1 densities, and action potential profile were found. We have the first demonstration that iPSC-CMs reprogrammed from skin fibroblasts of DMD patients with cardiomyopathy have a Na V1. 5-Kir2. 1 channelosome dysfunction, leading to reduced cardiac excitability and conduction.

Source link: https://doi.org/10.1101/2022.01.25.477696

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* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions