Lethal arrhythmia modelling using iPS cardiomyocytes

Description

In collaboration with Kyoto University and Keio University, we have established several lines of iPS cells from patients with inherited arrhythmias including long QT syndrome, Brugada syndrome, idiopathic ventricular fibrillation, and progressive cardiac conduction defect. iPS technologies have enabled us to analyze the electrophysiological and biochemical properties of beating cardiomyocytes of the patients with lethal arrhythmias. These experiments will provide us significant insights into pathophysiology underlying many life-threatening diseases of unknown causes.

Collaborators

Publications

1. Egashira T, Yuasa S, Suzuki T, Aizawa Y, Yamakawa H, Matsuhashi T, Ohno Y, Tohyama S, Okata S, Seki T, Kuroda Y, Yae K, Hashimoto H, Tanaka T, Hattori F, Sato T, Miyoshi S, Takatsuki S, Murata M, Kurokawa J, Furukawa T, Makita N, Aiba T, Shimizu W, Horie M, Kamiya K, Kodama I, Ogawa S, Fukuda K. Disease characterization using LQTS-specific induced pluripotent stem cells. Cardiovasc Res 2012;95:419-429 (pdf)   Pubmed: 22739119
2. Okata S, Yuasa S, Suzuki T, Ito S, Makita N, Yoshida T, Lin M, Kurokawa J, Seki T, Egashira T, Aizawa Y, Kodaira M, Motoda C, Yozu G, Shimojima M, Hayashiji N, Hashimoto H, Kuroda Y, Tanaka A, Murata M, Aiba T, Shimizu W, Horie M, Kamiya K, Furukawa T, Fukuda K. Embryonic type Na+ channel β-subunit, SCN3B masks the disease phenotype of Brugada syndrome. Sci Rep. 6, 34198 (2016). doi:10.1038/srep34198, (pdf)
3. Yamamoto Y, Makiyama T, Harita T, Sasaki K, Wuriyanghai Y, Hayano M, Nishiuchi S, Kohjitani H, Hirose S, Chen J, Yokoi F, Ishikawa T, Ohno S, Chonabayashi K, Motomura H, Yoshida Y, Horie M, Makita N, Kimura T. Allele-specific Ablation Rescues Electrophysiological Abnormalities in a Human iPS Cell Model of Long-QT Syndrome with a CALM2 Mutation. Human Mole Genet. 2017;26(9):1670-1677.(pdf)