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Abstract #0691

Delayed morphological phenotype in R6/2 mice carrying longer fragments of the human Huntington’s disease gene shown by in vivo MR imaging and spectroscopy

Stephen J Sawiak1, Nigel I Wood1, T Adrian Carpenter1, and A Jennifer Morton1

1University of Cambridge, Cambridge, United Kingdom

Huntington’s disease is caused by an unstable gene carrying excessive polyglutamine CAG repeats. Patients with genes carrying more CAG repeats have a less favourable outcome. The R6/2 mouse has a fragment of the human HD gene with 100 CAG repeats. We compared mice carrying longer CAG repeats (250 and 350) with wildtype controls using high-resolution in vivo longitudinal MRI and spectroscopy. Paradoxically, the 350CAG mice live longer, with ultimately similar but much slower atrophy and metabolic changes than 250CAG mice. They may, therefore, be a more useful model of HD with a longer window to evaluate pathology and treatments.

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