Gait analysis in a Mecp2 knockout mouse model of Rett syndrome reveals early-onset and progressive motor deficits

Ross, P. D., Gadalla, K. K. E. , Riddell, J. S., Bailey, M. E. S. and Cobb, S. R. (2014) Gait analysis in a Mecp2 knockout mouse model of Rett syndrome reveals early-onset and progressive motor deficits. PLoS ONE, 9(11), e112889. (doi:10.1371/journal.pone.0112889) (PMID:25392929)

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Abstract

Rett syndrome (RTT) is a genetic disorder characterized by a range of features including cognitive impairment, gait abnormalities and a reduction in purposeful hand skills. Mice harbouring knockout mutations in the Mecp2 gene display many RTT-like characteristics and are central to efforts to find novel therapies for the disorder. As hand stereotypies and gait abnormalities constitute major diagnostic criteria in RTT, it is clear that motor and gait-related phenotypes will be of importance in assessing preclinical therapeutic outcomes. We therefore aimed to assess gait properties over the prodromal phase in a functional knockout mouse model of RTT. In male Mecp2 knockout mice, we observed alterations in stride, coordination and balance parameters at 4 weeks of age, before the onset of other overt phenotypic changes as revealed by observational scoring. These data suggest that gait measures may be used as a robust and early marker of Mecp2-dysfunction in future preclinical therapeutic studies.

Item Type:Articles
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:Cobb, Dr Stuart and Gadalla, Dr Kamal and Bailey, Dr Mark and Riddell, Dr John
Authors: Ross, P. D., Gadalla, K. K. E., Riddell, J. S., Bailey, M. E. S., and Cobb, S. R.
College/School:College of Medical Veterinary and Life Sciences > Institute of Neuroscience and Psychology
College of Medical Veterinary and Life Sciences > School of Life Sciences
Journal Name:PLoS ONE
Publisher:Public Library of Science
ISSN:1932-6203
ISSN (Online):1932-6203
Copyright Holders:Copyright © 2014 The Authors
First Published:First published in PLoS ONE 9(11):e112889
Publisher Policy:Reproduced under a Creative Commons License

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