Altered neocortical dynamics in a mouse model of Williams–Beuren Syndrome



Downloads per month over past year

Dasilva, Miguel and Navarro-Guzman, Alvaro and Ortiz-Romero, Paula and Camassa, Alessandra and Muñoz-Cespedes, Alberto and Campuzano, Victoria and Sanchez-Vives, María V. (2019) Altered neocortical dynamics in a mouse model of Williams–Beuren Syndrome. Molecular Neurobiology . pp. 1-13. ISSN 0893-7648, ESSN: 1559-1182

[thumbnail of Dasilva, M. et al. 2019. Altered Neocortical Dynamics....pdf]
Creative Commons Attribution.


Official URL:


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.

Item Type:Article
Uncontrolled Keywords:Up states; Slow oscillations; Synchronization; Cerebral cortex; Cortical dynamics; Cognitive deficit
Subjects:Medical sciences > Biology > Neurosciences
ID Code:57290
Deposited On:09 Oct 2019 10:17
Last Modified:03 Jun 2021 11:03

Origin of downloads

Repository Staff Only: item control page