Abstract #0586

Distinct patterns of microstructural changes in mouse models of ALS/FTD with mutations in Tardbp, Fus and C9orf72

Aurea Martins Bach¹, Cristiana Tisca¹, Mohamed Tachrount¹, Carmelo Milioto^2,3, Mireia Carcolé^2,3, Shoshana Spring⁴, Remya R. Nair^5,6, Thomas J. Cunningham^6,7, Elizabeth M. C. Fisher⁸, Adrian M. Isaacs^2,3, Brian J. Nieman⁴, Jason Lerch¹, and Karla Miller¹

¹Wellcome Centre for Integrative Neuroimaging, FMRIB, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, United Kingdom, ²UK Dementia Research Institute at UCL, Faculty of Brain Sciences, University College London, London, United Kingdom, ³Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, University College London, London, United Kingdom, ⁴Mouse Imaging Centre, The Hospital for Sick Children, Toronto, ON, Canada, ⁵Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford, United Kingdom, ⁶Mammalian Genetics Unit, MRC Harwell Institute, Oxford, United Kingdom, ⁷MRC Prion Unit and Institute of Prion diseases, University College London, London, United Kingdom, ⁸Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, University College London, London, United Kingdom

Synopsis

Keywords: Neurodegeneration, PreclinicalAmyotrophic lateral sclerosis (ALS) and Frontotemporal dementia (FTD) form a disease spectrum with shared clinical, pathological and genetic features. The most common mutations in ALS/FTD are in the genes C9Orf72, TARDBP and FUS. We used post-mortem diffusion kurtosis imaging to assess microstructure imaging phenotypes in mouse models of ALS/FTD with mutations in these genes. While mice with mutation in Tardbp presented reduced FA and MO in various white matter tracts, no difference could be detected in mice with mutation in Fus, and increased MO in a portion of the corpus callosum was observed in mice with mutation in C9orf72.

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