Publication
Title
Single-cell and neuronal network alterations in an in vitro model of Fragile X syndrome
Author
Abstract
The Fragile X mental retardation protein (FMRP) is involved in many cellular processes and it regulates synaptic and network development in neurons. Its absence is known to lead to intellectual disability, with a wide range of comorbidities including autism. Over the past decades, FMRP research focused on abnormalities both in glutamatergic and GABAergic signaling, and an altered balance between excitation and inhibition has been hypothesized to underlie the clinical consequences of absence of the protein. Using Fmrp knockout mice, we studied an in vitro model of cortical microcircuitry and observed that the loss of FMRP largely affected the electrophysiological correlates of network development and maturation but caused less alterations in single-cell phenotypes. The loss of FMRP also caused a structural increase in the number of excitatory synaptic terminals. Using a mathematical model, we demonstrated that the combination of an increased excitation and reduced inhibition describes best our experimental observations during the ex vivo formation of the network connections.
Language
English
Source (journal)
Cerebral cortex. - New York
Publication
New York : 2020
ISSN
1047-3211
DOI
10.1093/CERCOR/BHZ068
Volume/pages
30 :1 (2020) , p. 31-46
ISI
000515101400003
Pubmed ID
30958540
Full text (Publisher's DOI)
Full text (open access)
Full text (publisher's version - intranet only)
UAntwerpen
Faculty/Department
Research group
Project info
The peripheral component of neurodegeneration: uptake and transmission of amyloid proteins in the enteric nervous system.
Human Brain Project Specific Grant Agreement 2 (HBP SGA2).
GENOMED - Genomics in Medicine.
Brain stimulation in the age of "light": Controlling and steering cortical cellular electrophysiology for future smart neuroprosthetics employing Optogenetics.
Publication type
Subject
Affiliation
Publications with a UAntwerp address
External links
Web of Science
Record
Identifier
Creation 05.06.2019
Last edited 24.11.2024
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