Establishment and Characterization of Induced Pluripotent (iPS) Stem Cells Derived from Immortalized B Cells of Cardiac Channelopathy Patients

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  • 先天性心疾患における不死化B細胞株由来induced pluripotent stem（iPS）細胞の樹立

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Abstract

Our lab has identified the mutated genes responsible for congenital heart defects (CHD) using immortalized B cell lines established from the patient’s blood. Using animal models, we have investigated how the mutations cause disease. However, because of the progress in induced pluripotent stem (iPS) cell technology, it would be better for us to use iPS cell-derived cardiac cells instead of mouse models. Therefore, we have established iPS cells from patients with long QT syndrome (LQTS) and sick sinus syndrome (SSS) and from normal controls. All established iPS cells expressed octamer-binding transcription factor-4 (Oct4), T-cell receptor alpha-1-60 (TRA-1-60), stage-specific embryonic antigen-4 (SSEA4), and Nanog genes and proteins and were alkaline phosphatase-positive. The mutated genes were not altered after reprogramming. Moreover, Epstein–Barr (EB) viral genes were not expressed after reprogramming. These results suggest that cardiac cells, such as cardiomyocytes, derived from iPS cells reflect the mutations of specific diseases and have no artifacts from the EB virus. Therefore, iPS cell-derived cardiac cells can be used as a powerful tool in in vitro disease models.

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