Torsten Ruest1, Julia M. Edgar2, William Matthew Holmes1, jennifer a. Barrie2, Klaus a. Nave3, thomas j. Anderson2, Debbie Dewar1
1Clinical Neuroscience, Faculty of Medicine, University of Glasgow, Glasgow, Scotland, UK; 2Applied Neurobiology Group, Faculty of Veterinary Medicine, University of Glasgow, UK; 3Dept of Neurogenetics, Max Planck Institute of Experimental Medicine, Goettingen, Germany
Duplication of the Plp1 gene is the most common cause of Pelizaeus-Merzbacher Disease (PMD), a disease characterised by dysmyelination. We investigated white matter abnormalities in a mouse model of PMD, inwhich the Plp1 gene is overexpressed, by correlating DTI measures with electron microscopy and immunohistochemical analyses of white matter changes. High-resolution 3-D DTI of contrast enhanced perfusion fixed mouse brains, were registered to a template to create average WT and Plp1-overexpresser datasets. Difference maps were generated and a group-wise, cluster based statistical analysis was performed. . The loss of anisotropy and increase in mean-water diffusion correlated with the severity of hypomyelination.
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