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Published March 9, 2019 | Version v1
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Data from: Influence of full-length dystrophin on brain volumes in mouse models of Duchenne muscular dystrophy

Authors/Creators

  • 1. Leiden University Medical Center
  • 2. University of Amsterdam

Description

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.

Notes

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].
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.

Files

MRI data part 1.zip

Files (1.7 GB)

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md5:dbfca8da62fca0648b460fef79a58190
639.9 MB Preview Download
md5:844488280effcd9b615838aefc48849a
1.1 GB Preview Download
md5:6408bab7b9d1d846ff6799feaa93d14f
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Additional details

Related works

Is cited by
10.1371/journal.pone.0194636 (DOI)