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Abstract #1166

DTI of a Mouse Model of Pelizaeus-Merzbacher Disease: Correlating MR Measures with Morphometric Analyses.

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.