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Lookup NU author(s): Dr Ingrid Verhaart,
Professor Annemieke Aartsma-Rus
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0).
Lack of dystrophin results in skeletal muscle dystrophy and dilated cardiomyopathy in humans and animal models. To achieve a basic understanding of the natural development of cardiomyopathy in different dystrophinopathy mouse models, left and right ventricular heart function was assessed at different ages in three dystrophinopathy mouse models (mdx, mdx/utrn+/- model and mdx/utrn-/-) using magnetic resonance imaging. Left ventricular function was significantly decreased, already at 2 months in the most severely affected mdx/utrn-/- mice. Furthermore, whereas heart function was stable in wild-type mice over time, both mdx and mdx/utrn+/- showed a clear decrease at 10 months of age, most prominently in the right ventricle. Therefore magnetic resonance imaging is an adequate technique to determine heart function in dystrophinopathy mouse models and can be used to assess the effect of potential therapies.
Author(s): Verhaart IE, van Duijn RJ, den Adel B, Roest AA, Verschuuren JJ, Aartsma-Rus A, van der Weerd L
Publication type: Article
Publication status: Published
Journal: Neuromuscular Disorders
Print publication date: 01/05/2012
Online publication date: 29/12/2011
Date deposited: 12/07/2016
ISSN (print): 0960-8966
ISSN (electronic): 1873-2364
PubMed id: 22209498
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