Key result
Dynamic substrate stiffening selectively triggers hypertrophic remodeling and sarcomere disarray in MYBPC3fs cardiomyocytes.
Why the study?
Existing in vitro models of hypertrophic cardiomyopathy rely on systems with static mechanical properties, precluding investigation of how dynamic mechanical loading interacts with genetic susceptibility during disease initiation.
Does dynamic mechanical substrate stiffening via a tunable magnetorheological elastomer platform unmask genotype-specific pathological remodeling in human iPSC-derived cardiomyocytes harboring an HCM-associated MYBPC3 frameshift variant?
Does dynamic mechanical substrate stiffening via a tunable magnetorheological elastomer platform unmask genotype-specific pathological remodeling in human iPSC-derived cardiomyocytes harboring an HCM-associated MYBPC3 frameshift variant?
Answers are grounded in this paper and cite their sources.
Dynamically tunable magnetorheological elastomer substrates provide a human-relevant in vitro platform demonstrating that the HCM-associated MYBPC3fs variant confers heightened mechanosensitivity to progressive mechanical stiffening.
Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease. While sarcomeric genomic variants drive HCM, a role for mechanical stress in disease pathogenesis is increasingly appreciated. However, existing in vitro models of HCM rely on model systems (e.g., hydrogels) with static mechanical properties, precluding investigation of how dynamic mechanical loading interacts with genetic susceptibility during disease initiation. Here, we leveraged a dynamically tunable magnetorheological elastomer (MRE) platform to model progressive mechanical stiffening in order to study mechano-genetic interactions in human induced pluripotent stem cell (hiPSC) derived cardiomyocytes harboring an HCM-associated MYBPC3 frameshift (MYBPC3fs) loss-of-function variant. Dynamic stiffening selectively unmasked pathological structural remodeling in MYBPC3fs cardiomyocytes while producing only modest changes in isogenic controls, suggesting that MYBPC3fs confers heightened mechanosensitivity. In MYBPC3fs cardiomyocytes, mechanical stiffening drove hypertrophic remodeling, sarcomere disorganization, nuclear remodeling, dysregulated NFAT3 signaling, and aberrant cell-cycle activity. MRE stiffening also drove fibroblast-to-myofibroblast transition in primary cardiac fibroblasts, recapitulating early fibrotic remodeling. Together, these findings position dynamically tunable MRE substrates as a human-relevant platform for dissecting mechano-genetic disease mechanisms and evaluating therapies targeting mechanotransduction pathways in HCM.
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Kooh et al. (2026) studied Hypertrophic cardiomyopathy (HCM). Dynamic substrate stiffening via tunable magnetorheological elastomer (MRE) platform (66 wt% carbonyl iron particle loading) vs. Unmagnetized MRE substrates and isogenic wild-type control cardiomyocytes was evaluated on Pathological cellular and structural remodeling (cell area, circularity, sarcomere organization, nuclear remodeling, and NFAT3 signaling). Dynamic magnetorheological elastomer stiffening selectively induced hypertrophic remodeling, sarcomere disorganization, nuclear remodeling, and elevated NFAT3 levels in MYBPC3fs cardiomyocytes.
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