Article
Readthrough-Promoting Drugs Gentamicin and PTC124 Fail to Rescue Nav1.5 Function of Human-Induced Pluripotent Stem Cell-Derived Cardiomyocytes Carrying Nonsense Mutations in the Sodium Channel Gene SCN5A.
Circulation. Arrhythmia and electrophysiology - 1 Nov 2016
Kosmidis Georgios, Veerman Christiaan C, Casini Simona, Verkerk Arie O, van de Pas Simone, Bellin Milena, Wilde Arthur A M, Mummery Christine L, Bezzina Connie R
Abstract excerpt
BACKGROUND: Several compounds have been reported to induce translational readthrough of premature stop codons resulting in the production of full-length protein by interfering with ribosomal proofreading. Here we examined the effect of 2 of these compounds, gentamicin and PTC124, in human-induced pluripotent stem cell (hiPSC)-derived cardiomyocytes bearing nonsense mutations in the sodium channel gene SCN5A,...
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