Murine models of ARVC: what have we learned and where do we go? Insight for therapeutics.

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Murine models of ARVC: what have we learned and where do we go? Insight for therapeutics.

Mostrar el registro sencillo del ítem Padrón Barthe, Laura Domínguez, Fernando García Pavía, Pablo Lara Pezzi, Enrique 2018-01-03T11:50:57Z 2018-01-03T11:50:57Z 2017
dc.identifier.issn 1435-1803 spa
dc.description.abstract Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare genetically-determined cardiac heart muscle disorder characterized by fibro-fatty replacement of the myocardium that causes heart failure and sudden cardiac death (SCD), predominantly in young males. The disease is often caused by mutations in genes encoding proteins of the desmosomal (cell-to-cell adhesion) complex, with a significant minority caused by mutations in non-desmosomal proteins. Existing treatment options are based on SCD prevention with the implantable cardioverter defibrillator and anti-heart failure medication. Heart transplantation may also be required and there is currently no cure. Several transgenic animal models have been developed to characterize the disease, assess its progression and determine the influence of potential environmental factors. Transgenic models are also very valuable for translational therapeutic approaches, to screen new treatment options that prevent and/or regress the disease. Here we review the available ARVC animal models reported to date, highlighting the most important pathophysiological findings and discussing the effect of treatments tested so far in this setting. We also describe gaps in current disease knowledge with the goal of facilitating research and improving patient outcomes. eng
dc.language.iso eng spa
dc.publisher Basic Research in Cardiology spa
dc.rights Atribución-NoComercial-SinDerivadas 3.0 España *
dc.rights.uri *
dc.subject ARVC spa
dc.subject Murine models spa
dc.subject Exercise spa
dc.subject Therapies spa
dc.subject Desmosomes spa
dc.title Murine models of ARVC: what have we learned and where do we go? Insight for therapeutics. spa
dc.type article spa
dc.description.version pre-print spa
dc.rights.accessRights openAccess spa
dc.description.extent 396 KB spa

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