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Behavioural impairments in mice of a novel FUS transgenic line recapitulate features of frontotemporal lobar degeneration.

Lysikova, Ekaterina A, Kukharsky, Michail S, Chaprov, Kirill D, Vasilieva, Nataliia A, Roman, Andrei Y, Ovchinnikov, Ruslan K, Deykin, Alexey V, Ninkina, Natalia and Buchman, Vladimir L 2019. Behavioural impairments in mice of a novel FUS transgenic line recapitulate features of frontotemporal lobar degeneration. Genes, Brain and Behavior 10.1111/gbb.12607
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Abstract

Multiple clinical and experimental evidence suggest that ALS and FTLD are members of a disease continuum. Pathological FUS inclusions have been observed in subsets of patients with these diseases but their anatomical distribution is different for two diseases. These structures are present in motor neurons in ALS cases but in cortical neurons in FTLD cases. Expression of a C‐terminally truncated form of human FUS causes an early onset and progressive motor neuron pathology in transgenic mice but only when these neurons express a certain level of this protein. Severe motor dysfunction and early lethality of mice with expression above this level prevent their use for studies of FTLD‐related pathology caused by expression of this form of FUS. In the present study we used another line of mice expressing the same protein but not developing any signs of motor system dysfunction due to substantially lower level of transgene expression in motor neurons. In a set of tests 5‐month old mice displayed certain behavioural abnormalities, including increased impulsivity, decreased anxiety and compromised social interaction, that recapitulate behaviour characteristics typically seen in FTLD patients.

Item Type: Article
Date Type: Published Online
Status: In Press
Schools: Biosciences
Publisher: Wiley
ISSN: 1601-1848
Date of First Compliant Deposit: 22 August 2019
Date of Acceptance: 19 August 2019
Last Modified: 18 Oct 2019 06:31
URI: http://orca.cf.ac.uk/id/eprint/125070

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