Abstract #1922

Altered brain structure in a mouse model of ALS with mutation in Tardbp is observed from early adulthood

Aurea Martins Bach¹, Shoshana Spring², Zeinab Ali³, Brian J. Nieman², John Sled², Remya R. Nair^3,4, Elizabeth Fisher⁵, Silvia Corrochano⁶, Thomas Cunningham^3,7, Jason Lerch¹, and Karla 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, Oxford, United Kingdom, ⁴Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford, United Kingdom, ⁵Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, University College London, London, United Kingdom, ⁶Neurological Disorders Group, Hospital Clínico San Carlos, IdISSC, Madrid, Spain, ⁷MRC Prion Unit and Institute of Prion diseases, University College London, London, United Kingdom

Synopsis

Keywords: Neurodegeneration, PreclinicalAmyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by aggregates of TDP-43 in the brain. The TDP-M323K mouse model of ALS has a mutation in Tardbp and presents progressive motor, neurological and behavioural phenotypes, in addition to widespread changes in brain volume at 12 months of age. Here, we assessed if these volumetric changes are progressive or if they are already present before other symptoms start to present. Post-mortem structural MRI in 3- and 12 months-old TDP-M323K mice revealed that brain volume is already altered in young adults despite the absence of major clinical and pathological phenotypes.

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