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Abstract #4459

Effect of dystrophin levels on brain volumes in Duchenne muscular dystrophy mouse models

Bauke Kogelman1, Artem Khmelinskii2,3, Maaike van Putten4, and Louise van der Weerd1,4

1Department of Radiology, Leiden University Medical Center, Leiden, Netherlands, 2Division of Image Processing, Dept. of Radiology, Leiden University Medical Center, Leiden, Netherlands, 3Percuros B.V., Enschede, Netherlands, 4Department of Human Genetics, Leiden University Medical Center, Leiden, Netherlands

Besides muscle, Duchenne muscular dystrophy also affects the brain, resulting in memory and behavior problems. The consequences of dystrophinopathy on gross macroscopic alterations in the mouse brain are unclear. We used a number of mouse models that express either 0%(mdx), 100%(Bl10-WT) or a low amount of dystrophin(mdx-Xist∆hs). We showed that while whole brain volume does not significantly differ between mdx and Bl10-WT mice, there are differences in volumes of individual brain substructures. These results are in line with human data, where brain volume was found to be reduced only in patients lacking both full-length dystrophin and the shorter isoform Dp140.

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