Please use this identifier to cite or link to this item:
Title: Altered neocortical dynamics in a mouse model of Williams-Beuren syndrome
Author: Dasilva, Miguel
Navarro Guzman, Alvaro
Ortiz Romero, Paula
Camassa, Alessandra
Muñoz Cespedes, Alberto
Campuzano Uceda, María Victoria
Sánchez-Vives, María Victoria
Keywords: Síndrome de Williams
Escorça cerebral
Trastorns de la cognició
Williams syndrome
Cerebral cortex
Cognition disorders
Issue Date: 30-Aug-2019
Publisher: Humana Press
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.
Note: Reproducció del document publicat a:
It is part of: Molecular Neurobiology, 2019, vol. 57, p. 765-777
Related resource:
ISSN: 0893-7648
Appears in Collections:Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer)

Files in This Item:
File Description SizeFormat 
692219.pdf2.83 MBAdobe PDFView/Open

This item is licensed under a Creative Commons License Creative Commons