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dc.contributor.authorConnell, James Wen
dc.contributor.authorAllison, Rachelen
dc.contributor.authorReid, Evanen
dc.date.accessioned2016-03-31T16:31:15Z
dc.date.available2016-03-31T16:31:15Z
dc.date.issued2016-03-28en
dc.identifier.citationConnell et al. PLOS ONE (2016) Vol. 11, Issue 3, Article e0152413. doi:10.1371/ journal.pone.0152413en
dc.identifier.issn1932-6203
dc.identifier.urihttps://www.repository.cam.ac.uk/handle/1810/254753
dc.description.abstractThe hereditary spastic paraplegias (HSPs) are genetic conditions in which there is progressive axonal degeneration in the corticospinal tract. Autosomal dominant mutations, including nonsense, frameshift and missense changes, in the gene encoding the microtubule severing ATPase spastin are the most common cause of HSP in North America and northern Europe. In this study we report quantitative gait analysis using a motorized treadmill system, carried out on mice knocked-in for a disease-associated mutation affecting a critical residue in the Walker A motif of the spastin ATPase domain. At 4 months and at one year of age homozygous mutant mice had a number of abnormal gait parameters, including in stride length and stride duration, compared to heterozygous and wild-type littermates. Gait parameters in heterozygous animals did not differ from wild-type littermates. We conclude that quantitative gait analysis using the DigiGait system sensitively detects motor abnormalities in a hereditary spastic paraplegia model, and would be a useful method for analyzing the effects of pharmacological treatments for HSP.
dc.description.sponsorshipThis work was supported by a grant from the United Kingdom Medical Research Council [MR/M00046X/1] awarded to ER, a Wellcome Trust Senior Research Fellow in Clinical Science grant awarded to ER [082381], and a grant from the Tom Wahlig Stiftung. The Cambridge Institute for Medical Research is supported by a Wellcome Trust Strategic Award [100140] a Wellcome Trust equipment grant [093026]. The Digigait equipment was purchased with support from the Marmaduke Shield Fund, University of Cambridge. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
dc.languageEnglishen
dc.language.isoenen
dc.publisherPLOS
dc.rightsAttribution 2.0 UK: England & Wales*
dc.rights.urihttp://creativecommons.org/licenses/by/2.0/uk/*
dc.titleQuantitative Gait Analysis Using a Motorized Treadmill System Sensitively Detects Motor Abnormalities in Mice Expressing ATPase Defective Spastinen
dc.typeArticle
dc.description.versionThis is the final version of the article. It first appeared from PLOS via http://dx.doi.org/10.1371/journal.pone.0152413en
prism.numbere0152413en
prism.publicationDate2016en
prism.publicationNamePLOS ONEen
prism.volume11en
dc.rioxxterms.funderMedical Research Council
dc.rioxxterms.funderWellcome Trust
dc.rioxxterms.projectidMR/M00046X/1
dc.rioxxterms.projectid082381 .
dc.rioxxterms.projectid100140
dc.rioxxterms.projectid093026
dcterms.dateAccepted2016-03-14en
rioxxterms.versionofrecord10.1371/journal.pone.0152413en
rioxxterms.licenseref.urihttp://www.rioxx.net/licenses/all-rights-reserveden
rioxxterms.licenseref.startdate2016-03-28en
dc.contributor.orcidReid, Evan [0000-0003-1623-7304]
dc.identifier.eissn1932-6203
rioxxterms.typeJournal Article/Reviewen
pubs.funder-project-idWellcome Trust (100140/Z/12/Z)
pubs.funder-project-idWellcome Trust (093026/Z/10/Z)
pubs.funder-project-idWellcome Trust (082381/Z/07/Z)
pubs.funder-project-idMRC (MR/M00046X/1)


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Attribution 2.0 UK: England & Wales
Except where otherwise noted, this item's licence is described as Attribution 2.0 UK: England & Wales