IPSC derived cardiomyocytes offer great promise for generating new mechanistic insight into diseases and enabling assessment of drug efficacy and safety for individual patients than currently used preclinical models. The European Bank for induced Pluripotent Stem Cells (EBiSC) iPSCs from individuals diagnosed with long QT syndrome, Brugada disease and hypertrophic cardiomyopathy with known disease associated genotypes and age-sex matched controls are available to support this research.
Current therapeutic strategies for a variety of inherited cardiac conditions are mostly symptomatic and they neither take into account phenotypic and genetic disease heterogeneity nor inter-individual differences in the response to drugs. Cardiomyocytes derived from disease-specific iPSCs represent a novel in vitro system with an unprecedented potential for generating new mechanistic insight into diseases, pathophysiology and drug efficacy and safety. High quality iPSC lines from patients with genetic heart diseases for in vitro use includes cell lines derived from patients with long QT syndrome type 1, long QT syndrome type 2 (UKKi018-A,B,C; UKKi019-A,B,C and UKKi023-A,B,C lines carrying different KCNH2 mutations), Brugada syndrome (UKKi024-A,B,C line carrying a SCN5A mutation) and hypertrophic cardiomyopathy (UKKi025- A,B,C line carrying a MYH7 mutation).
All lines were generated from donated blood cells using Sendai CytoTune under feeder-free conditions. They underwent extensive characterization to ensure quality in terms of sterility, viability, identity, pluripotency, genetic stability, and absence of HIV, HBV, HCV and reprogramming vectors. This data and the associated clinical information are accessible on a searchable public registry for human pluripotent stem cell lines, hPSCreg. These iPS cell lines represent a valuable resource of lines for the study of disease and the development of new treatments. EBiSC2 aims to simplify cardiac disease research and is working to provide iPSC derived Cardiomyocytes directly to users.
Written by Rachel Steeg, The European Bank for induced Pluripotent Stem Cells (EBiSC)
Image courtesy of Tomo Šarić, Medical Faculty, University of Cologne, Germany. Cardiomyocytes derived from iPSC line of a patient with hypertrophic cardiomyopathy carrying a mutation in MYH7 gene encoding a myosin heavy chain beta (MHC-β) isoform. Red indicates the muscle protein alpha-actinin and blue the cell nuclei.
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