{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Marksteiner J"],"funding":["Austrian Science Fund FWF","Ludwig Boltzmann Gesellschaft","Ludwig Boltzmann Gesellschaft (LBG)"],"pagination":["H648-H660"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC7618084"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["329(3)"],"pubmed_abstract":["Cardiac arrhythmias significantly contribute to mortality in Duchenne muscular dystrophy (DMD), a severe muscle disease caused by dystrophin deficiency. Using the <i>mdx</i> mouse model for human DMD, we previously showed that the lack of dystrophin induces a significant loss of peak sodium current (<i>I</i><sub>Na</sub>) in ventricular cardiomyocytes. This provided a mechanistic explanation for ventricular conduction defects and concomitant arrhythmias in the dystrophic heart. The extracellular matrix protein tenascin C (TN-C), a major remodeling factor in the diseased heart, is strongly upregulated in DMD. The consequences of TN-C upregulation in the dystrophic heart, however, are unknown. Here, we tested if TN-C induces electrical remodeling in the dystrophic heart, and if inhibition of"],"journal":["American journal of physiology. Heart and circulatory physiology"],"pubmed_title":["Inhibition of tenascin C rescues abnormally reduced Na currents in dystrophin-deficient ventricular cardiomyocytes."],"pmcid":["PMC7618084"],"funding_grant_id":["P 35878","P 35542","CARREM Project Line","2020-2024","P35878-B","P35542-B"],"pubmed_authors":["Hackl B","Szabo PL","Schicker K","Kiss A","Koenig X","David FO","Schindler N","Dostal C","Hilber K","Todt H","Podesser BK","Sauer J","Lilliu E","Marksteiner J","Hohenegger M"],"additional_accession":[]},"is_claimable":false,"name":"Inhibition of tenascin C rescues abnormally reduced Na currents in dystrophin-deficient ventricular cardiomyocytes.","description":"Cardiac arrhythmias significantly contribute to mortality in Duchenne muscular dystrophy (DMD), a severe muscle disease caused by dystrophin deficiency. Using the <i>mdx</i> mouse model for human DMD, we previously showed that the lack of dystrophin induces a significant loss of peak sodium current (<i>I</i><sub>Na</sub>) in ventricular cardiomyocytes. This provided a mechanistic explanation for ventricular conduction defects and concomitant arrhythmias in the dystrophic heart. The extracellular matrix protein tenascin C (TN-C), a major remodeling factor in the diseased heart, is strongly upregulated in DMD. The consequences of TN-C upregulation in the dystrophic heart, however, are unknown. Here, we tested if TN-C induces electrical remodeling in the dystrophic heart, and if inhibition of","dates":{"release":"2025-01-01T00:00:00Z","publication":"2025 Sep","modification":"2026-06-02T20:28:18.099Z","creation":"2026-04-20T03:09:59.714Z"},"accession":"S-EPMC7618084","cross_references":{"pubmed":["40789177"],"doi":["10.1152/ajpheart.00307.2025"]}}