ArticleBioengineering & translational medicine2026
Pediatric dilated cardiomyopathy caused by TNNT2-R151W mutation: Modeling and rescue in patient-derived induced pluripotent stem cells and engineered heart tissue.
Article in Bioengineering & translational medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Dilated cardiomyopathy (DCM) is the most prevalent pediatric cardiomyopathy and has a poor prognosis. Although heart transplantation is the only curative option, the severe donor shortage in Japan underscores an urgent need for alternative therapies. Here, we generated induced pluripotent stem cell (iPSC) lines from two unrelated children with the same TNNT2-R151W mutation. We then investigated the cellular characteristics of the cardiomyocytes derived from these iPSC lines (R151W iPSC-CMs) and evaluated their contractile function in pillar-based engineered heart tissue (EHT). We also assessed the therapeutic potential of overexpressing wild-type TNNT2 in patient-derived iPSCs (wtTNNT2-OE-iPSCs). R151W-iPSC-CMs exhibited pronounced sarcomere disarray, attenuated Ca
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