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Assessment of cardiac function in three mouse dystrophinopathies by magnetic resonance imaging

Lookup NU author(s): Dr Ingrid Verhaart, Professor Annemieke Aartsma-Rus

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Abstract

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.

Publication metadata

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

Year: 2012

Volume: 22

Issue: 5

Pages: 418-426

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

Publisher: Elsevier

URL: http://dx.doi.org/10.1016/j.nmd.2011.10.025

DOI: 10.1016/j.nmd.2011.10.025

PubMed id: 22209498

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