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Midwest Pediatric Cardiology Society




Marfan syndrome (MFS) represents a genetic disorder with variable phenotypic expression. The main cardiovascular sequelae of MFS include aortic aneurysm/dissection and cardiomyopathy. While significant advances in the understanding of TGF-β signaling have led to promising therapeutic targets for the treatment of aortopathy, clinical studies have tempered this optimism. In particular, these studies suggest additional signaling pathways that play a significant role in aortic disease progression. Furthermore, even less is known with respect to effectors involved in MFS-induced cardiomyopathy. To date, studies aimed at elucidating molecular mechanisms involved in MFS-induced disease progression have been hampered by the lack of an accelerated disease model. Meanwhile, transient receptor potential (TRP) channels have been implicated as key effectors in other vascular smooth muscle and cardiomyocyte pathologies, yet there is a paucity of investigation focused on their involvement in the aortopathy and cardiomyopathy of MFS. In these studies, we investigate the importance of TRP channels in the pathogenesis of MFS-induced aortopathy and cardiomyopathy by evaluating differential expression in a novel murine model of accelerated MFS etiology

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