Show simple item record

dc.contributor.authorLione, Lisa
dc.contributor.authorCarter, R. J.
dc.contributor.authorHumby, T.
dc.contributor.authorMangiarini, L.
dc.contributor.authorMahal, A.
dc.contributor.authorBates, G. P.
dc.contributor.authorDunnett, S.B.
dc.contributor.authorMorton, A. J.
dc.date.accessioned2013-07-03T15:17:05Z
dc.date.available2013-07-03T15:17:05Z
dc.date.issued1999-04-15
dc.identifier.citationLione , L , Carter , R J , Humby , T , Mangiarini , L , Mahal , A , Bates , G P , Dunnett , S B & Morton , A J 1999 , ' Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation ' , Journal of Neuroscience , vol. 19 , no. 8 , pp. 3248-3257 .
dc.identifier.issn0270-6474
dc.identifier.otherPURE: 670696
dc.identifier.otherPURE UUID: 0a926148-7141-4008-8f66-7f8c64c4541d
dc.identifier.otherWOS: 000079568300036
dc.identifier.otherScopus: 0033560924
dc.identifier.urihttp://hdl.handle.net/2299/11081
dc.description.abstractTransgenic mice expressing exon 1 of the human Huntington's disease (HD) gene carrying a 141-157 CAG repeat (line R6/2) develop a progressive neurological phenotype with motor symptoms resembling those seen in HD. We have characterized the motor deficits in R6/2 mice using a battery of behavioral tests selected to measure motor aspects of swimming, fore- and hindlimb coordination, balance, and sensorimotor gating [swimming tank, rotarod, raised beam, fore- and hindpaw footprinting, and acoustic startle/prepulse inhibition (PPI)I. Behavioral testing was performed on female hemizygotic R6/2 transgenic mice (n = 9) and female wild-type littermates (n = 22) between 5 and 14 weeks of age. Transgenic mice did not show an overt behavioral phenotype until around 8 weeks of age. However, as early as 5-6 weeks of age they had significant difficulty swimming, traversing the narrowest square (5 mm) raised beam, and maintaining balance on the rotarod at rotation speeds of 33-44 rpm. Furthermore, they showed significant impairment in prepulse inhibition tan impairment also seen in patients with HD). Between 8 and 15 weeks, R6/2 transgenic mice showed a progressive deterioration in performance on all of the motor tests. Thus R6/2 mice show measurable deficits in motor behavior that begin subtly and increase progressively until death. Our data support the use of R6/2 mice as a model of HD and indicate that they may be useful for evaluating therapeutic strategies for HD, particularly those aimed at reducing the severity of motor symptoms or slowing the course of the disease.en
dc.format.extent10
dc.language.isoeng
dc.relation.ispartofJournal of Neuroscience
dc.subjecttransgenic mice
dc.subjectHuntington's disease
dc.subjectCAG repeat
dc.subjectmotor behavior
dc.subjectprepulse inhibition
dc.subjectsensorimotor gating
dc.subjectpolyglutamine repeat diseases
dc.subjectTOXIN 3-NITROPROPIONIC ACID
dc.subjectCAG REPEAT
dc.subjectPREPULSE INHIBITION
dc.subjectQUINOLINIC ACID
dc.subjectABNORMALITIES
dc.subjectMODEL
dc.subjectDISORDERS
dc.subjectLESIONS
dc.subjectGENE
dc.subjectRATS
dc.titleCharacterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutationen
dc.contributor.institutionDepartment of Human and Environmental Sciences
dc.contributor.institutionSchool of Life and Medical Sciences
dc.contributor.institutionHealth & Human Sciences Research Institute
dc.contributor.institutionPharmacology and Clinical Science Research
dc.contributor.institutionCardiovascular Pathologies
dc.contributor.institutionDiabetic neuropathies
dc.contributor.institutionTRP Ion channels
dc.description.statusPeer reviewed
rioxxterms.typeJournal Article/Review
herts.preservation.rarelyaccessedtrue


Files in this item

FilesSizeFormatView

There are no files associated with this item.

This item appears in the following Collection(s)

Show simple item record