Assessment of cardiac function in three mouse dystrophinopathies by magnetic resonance imaging.
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 2months 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 10months 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.
Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands.
Ingrid E C
IE
Verhaart
Rianne J M
RJ
van Duijn
Brigit
B
den Adel
Arno A W
AA
Roest
Jan J G M
JJ
Verschuuren
Annemieke
A
Aartsma-Rus
Louise
L
van der Weerd
D000818:Animals; D002311:Cardiomyopathy, Dilated / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D004195:Disease Models, Animal; D016189:Dystrophin / Q000172:deficiency / Q000235:genetics; D005355:Fibrosis / Q000235:genetics; D006352:Heart Ventricles / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D018482:Muscle, Skeletal / Q000473:pathology; D009137:Muscular Dystrophy, Animal / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D016277:Ventricular Function, Left
D016189:Dystrophin