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Activation of PDGFRA signaling contributes to filamin C–related arrhythmogenic cardiomyopathy

23 Feb 2022

Abstract excerpt

FLNC truncating mutations ( FLNCtv ) are prevalent causes of inherited dilated cardiomyopathy (DCM), with a high risk of developing arrhythmogenic cardiomyopathy. We investigated the molecular mechanisms of mutant FLNC in the pathogenesis of arrhythmogenic DCM (a-DCM) using patient-specific induced pluripotent stem cell–derived cardiomyocytes (iPSC-CMs). We demonstrated that iPSC-CMs from two patients with...

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PMID: 35196083
DOI: 10.1126/sciadv.abk0052

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