Please use this identifier to cite or link to this item: http://hdl.handle.net/2440/53197
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Type: Journal article
Title: Open field locomotor activity and anxiety-related behaviors in mucopolysaccharidosis type IIIA mice
Author: Lau, A.
Crawley, A.
Hopwood, J.
Hemsley, K.
Citation: Behavioural Brain Research, 2008; 191(1):130-136
Publisher: Elsevier Science BV
Issue Date: 2008
ISSN: 0166-4328
1872-7549
Statement of
Responsibility: 
Adeline A. Lau, Allison C. Crawley, John J. Hopwood and Kim M. Hemsley
Abstract: Mucopolysaccharidosis (MPS) IIIA, or Sanfilippo syndrome, is a lysosomal storage disorder characterized by severe and progressive neuropathology. Following an asymptomatic period, patients may present with sleep disturbances, cognitive decline, aggressive tendencies and hyperactivity. A naturally-occurring mouse model of MPS IIIA also exhibits many of these behavioral features and has been recently back-crossed onto a C57BL/6 genetic background. To more thoroughly characterize the behavioral phenotype of congenic MPS IIIA mice, we assessed exploratory activity and unconditioned anxiety-related behavior in the elevated plus maze (EPM) and open field locomotor activity. Although MPS IIIA male mice were less active in the EPM at 18 and 20 weeks of age, they were more likely to explore the open arms than their normal counter-parts suggesting reduced anxiety. Repeated EPM testing reduced exploration of the open arms in MPS IIIA mice. In the open field test, significant reductions in activity were evident in naïve-tested male MPS IIIA mice from 10 weeks of age. Female normal and MPS IIIA mice displayed similar exploratory activity in the open field test. These differences in anxiety and locomotor activity will allow us to evaluate the efficacy of therapeutic regimes for MPS IIIA as a forerunner to developing safe and effective therapies for Sanfilippo patients.
Keywords: Mucopolysaccharidosis; Sanfilippo syndrome; Elevated plus maze; Anxiety; Open field activity; C57BL/6
Description: Crown copyright © 2008 Published by Elsevier B.V.
RMID: 0020081076
DOI: 10.1016/j.bbr.2008.03.024
Appears in Collections:Paediatrics publications

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