Influence of full-length dystrophin on brain volumes in mouse models of Duchenne muscular dystrophy

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Abstract

© 2018 Kogelman et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Duchenne muscular dystrophy (DMD) affects besides muscle also the brain, resulting in memory and behavioral problems. The consequences of dystrophinopathy on gross macroscopic alterations are unclear. To elucidate the effect of full-length dystrophin expression on brain morphology, we used high-resolution post-mortem MRI in mouse models that either express 0% (mdx), 100% (BL10) or a low amount of full-length dystrophin (mdx-XistΔhs). While absence or low amounts of full-length dystrophin did not significantly affect whole brain volume and skull morphology, we found differences in volume of individual brain structures. The results are in line with observations in humans, where whole brain volume was found to be reduced only in patients lacking both full-length dystrophin and the shorter isoform Dp140.

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Kogelman, B., Khmelinskii, A., Verhaart, I., van Vliet, L., Bink, D. I., Aartsma-Rus, A., … van der Weerd, L. (2018). Influence of full-length dystrophin on brain volumes in mouse models of Duchenne muscular dystrophy. PLoS ONE, 13(3). https://doi.org/10.1371/journal.pone.0194636

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