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Abstract #0899

Brain structure in the homozygous FUSDelta14 mouse recapitulates amyotrophic lateral sclerosis phenotypes

Aurea B. Martins Bach1, Lily Qiu1, Jacob Ellegood2, Nick Wang2, Brian J. Nieman2, John G. Sled2, Remya R. Nair3, Elizabeth M. C. Fisher3,4, Thomas J. Cunningham3, Jason Lerch1, and Karla L. Miller1
1Wellcome Centre for Integrative Neuroimaging, FMRIB, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, United Kingdom, 2Mouse Imaging Centre, The Hospital for Sick Children, Toronto, ON, Canada, 3Mammalian Genetics Unit, MRC Harwell Institute, Oxfordshire, United Kingdom, 4Department of Neuromuscular Diseases, Institute of Neurology, University College London, London, United Kingdom

This study assesses changes in brain anatomy with MRI in the homozygous humanized FUSDelta14 mouse model of amyotrophic lateral sclerosis (ALS). Post-mortem brain T2w-images were acquired at 7T, with 40μm isotropic resolution. After registration, the deformation fields were compared between mutant and wild-type mice. Homozygous FUSDelta14 mice exhibited atrophy in multiple grey and white matter structures. These results are in agreement with observations such as cortical thinning and alterations in white matter microstructure in ALS patients. Homozygous humanized FUSDelta14 mice show an early brain phenotype and are therefore a promising model for the study of ALS pathogenic mechanisms.

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