MRI data part 1

Contains Bruker scan data (including localizer scans) as 2dseq files and can be easily read with Bruker ParaVision software. On request, data can be converted to Nifti/Dicom

MRI data part 2

Contains Bruker scan data (including localizer scans) as 2dseq files and can be easily read with Bruker ParaVision software. On request, data can be converted to Nifti/Dicom

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

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 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-Xist∆hs). While absence or low amounts of dystrophin did not result in significantly different whole brain volume and skull morphology, we found differences in volume of individual brain structures. The found 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

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

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 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-Xist∆hs). While absence or low amounts of dystrophin did not result in significantly different whole brain volume and skull morphology, we found differences in volume of individual brain structures. The found 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

Template and brain volumes

This zip file contains the following information: mouse brain template, brain ROIs, registration settings and the processed data. Each mouse brain was registered to a template mouse brain and volumes (whole brain + 22 structures) were extracted using the ROIs. Volumes are stored [per structure per mouse] and volumes are stored [per structure per strain]

Volumes (in mm³) of 22 brain structures in mouse models of Duchenne muscular dystrophy.

<p>The volumes of 22 segmented brain structures in <i>mdx</i>, BL10, <i>mdx</i>-<i>Xist</i>^Δhs and BL10-<i>Xist</i>^Δhs mice are presented in mm³. Body mass was measured just before sacrificing the mice, except for two <i>mdx-Xist</i>^Δhs mice of which body mass was not obtained. The volumes of these 22 structures were normalized to whole brain volume and compared using Welch’s T-tests within each genetic background (<i>mdx vs</i> BL10 and <i>mdx</i>-<i>Xist</i>^Δhs <i>vs</i> BL10-<i>Xist</i>^Δhs) and corrected for multiple comparisons using the false discovery rate. The corresponding <i>P</i>-values are presented and structures which are significantly different in volume are written in bold. The hippocampus, globus pallidus, caudate putamen and hypothalamus were different between <i>mdx</i> and BL10 mice. Thirteen structures were different between <i>mdx</i>-<i>Xist</i>^Δhs and BL10-<i>Xist</i>^Δhs mice, consisting of a mixture of both grey and white matter structures.</p

Dystrophin expression in <i>mdx-Xist</i>^Δhs mice.

<p><b>A:</b> Representative Western blot of <i>mdx-Xist</i>^Δhs brains revealed that mice expressed low levels of full-length dystrophin in their brain. Levels varied between individuals but were below 20% in all mice. Post Synaptic Density Protein 95 (Psdp95) served as a loading control. Wildtype samples for the concentration series were diluted in <i>mdx</i> protein lysate to ensure equal protein loading of all samples. <b>B:</b> mRNA expression levels of neither full-length nor Dp71 differed between <i>mdx</i> and BL10 mice. However, expression of Dp427c was higher in BL10 compared to <i>mdx</i>-<i>Xist</i>^Δhs mice. Error bars represents the standard deviation. Asterisk indicates <i>P</i><0.05.</p

The volumes of 22 segmented brain structures in <i>mdx</i>, BL10, <i>mdx</i>-<i>Xist</i>^Δhs and BL10-<i>Xist</i>^Δhs mice were compared within each genetic background (<i>mdx vs</i> BL10 and <i>mdx</i>-<i>Xist</i>^Δhs <i>vs</i> BL10-<i>Xist</i>^Δhs).

<p>The brain structures found significantly different in volume after false discovery rate correction are shown and consisted of both grey and white matter structures for both comparisons. Coronal, sagittal and axial planes are shown indicated by white lines. <b>A:</b> Comparison between <i>mdx</i> and BL10 showed four different brain structures: the hippocampus, globus pallidus, caudate putamen and hypothalamus. <b>B:</b> Comparison between <i>mdx</i>-<i>Xist</i>^Δhs and BL10-<i>Xist</i>^Δhs showed 13 different structures.</p