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Altered neocortical dynamics in a mouse model of Williams-Beuren syndrome

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dc.contributor.author Dasilva, Miguel
dc.contributor.author Navarro-Guzman, Alvaro
dc.contributor.author Ortiz Romero, Paula, 1994-
dc.contributor.author Camassa, Alessandra
dc.contributor.author Muñoz-Cespedes, Alberto
dc.contributor.author Campuzano Uceda, María Victoria
dc.contributor.author Sanchez-Vives, Maria V.
dc.date.accessioned 2019-10-10T10:55:10Z
dc.date.available 2019-10-10T10:55:10Z
dc.date.issued 2020
dc.identifier.citation Dasilva M, Navarro-Guzman A, Ortiz-Romero P, Camassa A, Muñoz-Cespedes A, Campuzano V et al. Altered neocortical dynamics in a mouse model of Williams-Beuren syndrome. Mol Neurobiol. 2020;57(2):765-73. DOI: 10.1007/s12035-019-01732-4
dc.identifier.issn 0893-7648
dc.identifier.uri http://hdl.handle.net/10230/42420
dc.description.abstract Williams-Beuren syndrome (WBS) is a rare neurodevelopmental disorder characterized by moderate intellectual disability and learning difficulties alongside behavioral abnormalities such as hypersociability. Several structural and functional brain alterations are characteristic of this syndrome, as well as disturbed sleep and sleeping patterns. However, the detailed physiological mechanisms underlying WBS are mostly unknown. Here, we characterized the cortical dynamics in a mouse model of WBS previously reported to replicate most of the behavioral alterations described in humans. We recorded the laminar local field potential generated in the frontal cortex during deep anesthesia and characterized the properties of the emergent slow oscillation activity. Moreover, we performed micro-electrocorticogram recordings using multielectrode arrays covering the cortical surface of one hemisphere. We found significant differences between the cortical emergent activity and functional connectivity between wild-type mice and WBS model mice. Slow oscillations displayed Up states with diminished firing rate and lower high-frequency content in the gamma range. Lower firing rates were also recorded in the awake WBS animals while performing a marble burying task and could be associated with the decreased spine density and thus synaptic connectivity in this cortical area. We also found an overall increase in functional connectivity between brain areas, reflected in lower clustering and abnormally high integration, especially in the gamma range. These results expand previous findings in humans, suggesting that the cognitive deficits characterizing WBS might be associated with reduced excitability, plus an imbalance in the capacity to functionally integrate and segregate information.
dc.description.sponsorship This work was supported by EU H2020 Research and Innovation Programme under Grant Agreement No. 720270 (HBP SGA2), BFU2017-85048-R Spanish Ministry of Science and CERCA Programme/Generalitat de Catalunya to MVSV.
dc.format.mimetype application/pdf
dc.language.iso eng
dc.publisher Springer
dc.relation.ispartof Molecular Neurobiology. 2020;57(2):765-73
dc.rights © The Author(s) 2019. Open Access. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.
dc.rights.uri http://creativecommons.org/licenses/by/4.0/
dc.title Altered neocortical dynamics in a mouse model of Williams-Beuren syndrome
dc.type info:eu-repo/semantics/article
dc.identifier.doi http://dx.doi.org/10.1007/s12035-019-01732-4
dc.subject.keyword Up states
dc.subject.keyword Slow oscillations
dc.subject.keyword Synchronization
dc.subject.keyword Cerebral cortex
dc.subject.keyword Cortical dynamics
dc.subject.keyword Cognitive deficit
dc.relation.projectID info:eu-repo/grantAgreement/EC/H2020/720270
dc.relation.projectID info:eu-repo/grantAgreement/ES/2PE/BFU2017-85048-R
dc.rights.accessRights info:eu-repo/semantics/openAccess
dc.type.version info:eu-repo/semantics/publishedVersion


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