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dc.contributor.authorPerentos, N.en
dc.contributor.authorMartins, A. Q.en
dc.contributor.authorWatson, T. C.en
dc.contributor.authorBartsch, U.en
dc.contributor.authorMitchell, Nadia L.en
dc.contributor.authorPalmer, David N.en
dc.contributor.authorJones, M. W.en
dc.contributor.authorMorton, A. J.en
dc.date.accessioned2017-12-14T02:17:09Z
dc.date.available2015-02-27en
dc.date.issued2015-04-01en
dc.date.submitted2014-12-12en
dc.identifier.citationPerentos et al. (2015). Translational neurophysiology in sheep: Measuring sleep and neurological dysfunction in CLN5 Batten disease affected sheep. Brain, 138(4), 862-874. doi:10.1093/brain/awv026en
dc.identifier.issn0006-8950en
dc.identifier.urihttps://hdl.handle.net/10182/8850
dc.description.abstractCreating valid mouse models of slowly progressing human neurological diseases is challenging, not least because the short lifespan of rodents confounds realistic modelling of disease time course. With their large brains and long lives, sheep offer significant advantages for translational studies of human disease. Here we used normal and CLN5 Batten disease affected sheep to demonstrate the use of the species for studying neurological function in a model of human disease. We show that electroencephalography can be used in sheep, and that longitudinal recordings spanning many months are possible. This is the first time such an electroencephalography study has been performed in sheep. We characterized sleep in sheep, quantifying characteristic vigilance states and neurophysiological hallmarks such as sleep spindles. Mild sleep abnormalities and abnormal epileptiform waveforms were found in the electroencephalographies of Batten disease affected sheep. These abnormalities resemble the epileptiform activity seen in children with Batten disease and demonstrate the translational relevance of both the technique and the model. Given that both spontaneous and engineered sheep models of human neurodegenerative diseases already exist, sheep constitute a powerful species in which longitudinal in vivo studies can be conducted. This will advance our understanding of normal brain function and improve our capacity for translational research into neurological disorders.en
dc.format.extent862-874en
dc.language.isoenen
dc.publisherOxford University Press on behalf of the Guarantors of Brainen
dc.relationThe original publication is available from - Oxford University Press on behalf of the Guarantors of Brain - https://doi.org/10.1093/brain/awv026en
dc.relation.urihttps://doi.org/10.1093/brain/awv026en
dc.rights© The Author (2015). Published by Oxford University Press on behalf of the Guarantors of Brain. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.comen
dc.rights.urihttp://creativecommons.org/licenses/by-nc/4.0/en
dc.rights.urihttp://creativecommons.org/licenses/by-nc/4.0/en
dc.subjectbrain atrophyen
dc.subjectlysosomal storage diseaseen
dc.subjectneurodegenerationen
dc.subjectexperimental modelsen
dc.subjectepilepsyen
dc.subjectseizuresen
dc.subjectneuronal ceroid lipofuscinosisen
dc.subjectNCLen
dc.subjectNeurology & Neurosurgeryen
dc.subject.meshAnimalsen
dc.subject.meshSheepen
dc.subject.meshHumansen
dc.subject.meshNervous System Diseasesen
dc.subject.meshDisease Models, Animalen
dc.subject.meshMembrane Proteinsen
dc.subject.meshSleepen
dc.subject.meshNeuronal Ceroid-Lipofuscinosesen
dc.subject.meshTranslational Medical Researchen
dc.titleTranslational neurophysiology in sheep: Measuring sleep and neurological dysfunction in CLN5 Batten disease affected sheepen
dc.typeJournal Article
lu.contributor.unitLincoln Universityen
lu.contributor.unitFaculty of Agriculture and Life Sciencesen
lu.contributor.unitDepartment of Wine, Food and Molecular Biosciencesen
lu.contributor.unitResearch Management Officeen
lu.contributor.unit/LU/Research Management Office/2018 PBRF Staff groupen
dc.identifier.doi10.1093/brain/awv026en
dc.subject.anzsrc060805 Animal Neurobiologyen
dc.subject.anzsrc1109 Neurosciencesen
dc.subject.anzsrc11 Medical And Health Sciencesen
dc.subject.anzsrc17 Psychology And Cognitive Sciencesen
dc.relation.isPartOfBrainen
pubs.issue4en
pubs.organisational-group/LU
pubs.organisational-group/LU/Agriculture and Life Sciences
pubs.organisational-group/LU/Agriculture and Life Sciences/WFMB
pubs.organisational-group/LU/Research Management Office
pubs.organisational-group/LU/Research Management Office/2018 PBRF Staff group
pubs.publication-statusPublisheden
pubs.volume138en
dc.identifier.eissn1460-2156en
dc.rights.licenceAttribution-NonCommercialen
dc.rights.licenceAttribution-NonCommercialen
lu.identifier.orcid0000-0002-9703-5351


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