Brugada syndrome (BrS) is an inherited arrhythmia disorder with limited drug therapy, quinidine being the principal agent. Loss-of-function variants in SCN5A reduce the cardiac sodium current (INa), but the arrhythmogenic imbalance also depends on the transient outward potassium current (Ito). Here we generated human i...
Y. Guéret, A. Girardeau, S. Godin et al.· medRxiv· 0 citations
Abstract Increased late sodium current (INaL) is a central mechanism underlying both inherited and acquired cardiac arrhythmias. Although Nav1.5 is the dominant cardiac sodium channel, multiple tetrodotoxin-sensitive (TTX-S) Nav isoforms are also expressed in cardiomyocytes, particularly within transverse tubules, wher...
Hugo Millet, M. Choteau-Bodor, T. Stervinou et al.· Europace· 0 citations
The findings support a model in which non-SCN5A BrS is associated with early dysregulation of developmental transcriptional networks controlling cardiac conduction and extracellular matrix organization, whereas SCN5A-mediated BrS primarily results from sodium channel deficiency with limited developmental remodeling.
T. Stervinou, B. Cimarosti, R. Canac et al.· medRxiv· 0 citations
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