Altered trajectories of neurodevelopment and behavior in mouse models of Rett syndrome.
Barrel field
Behavior
Developmental disorder
Glutamate receptors, review
MeCP2
Molecular biology
Morphometry
Neuropathology
Plasticity
Rett
Journal
Neurobiology of learning and memory
ISSN: 1095-9564
Titre abrégé: Neurobiol Learn Mem
Pays: United States
ID NLM: 9508166
Informations de publication
Date de publication:
11 2019
11 2019
Historique:
received:
30
03
2018
revised:
17
10
2018
accepted:
16
11
2018
pubmed:
7
12
2018
medline:
31
7
2020
entrez:
4
12
2018
Statut:
ppublish
Résumé
Rett Syndrome (RTT) is a genetic disorder that is caused by mutations in the x-linked gene coding for methyl-CpG-biding-protein 2 (MECP2) and that mainly affects females. Male and female transgenic mouse models of RTT have been studied extensively, and we have learned a great deal regarding RTT neuropathology and how MeCP2 deficiency may be influencing brain function and maturation. In this manuscript we review what is known concerning structural and coinciding functional and behavioral deficits in RTT and in mouse models of MeCP2 deficiency. We also introduce our own corroborating data regarding behavioral phenotype and morphological alterations in volume of the cortex and striatum and the density of neurons, aberrations in experience-dependent plasticity within the barrel cortex and the impact of MeCP2 loss on glial structure. We conclude that regional structural changes in genetic models of RTT show great similarity to the alterations in brain structure of patients with RTT. These region-specific modifications often coincide with phenotype onset and contribute to larger issues of circuit connectivity, progression, and severity. Although the alterations seen in mouse models of RTT appear to be primarily due to cell-autonomous effects, there are also non-cell autonomous mechanisms including those caused by MeCP2-deficient glia that negatively impact healthy neuronal function. Collectively, this body of work has provided a solid foundation on which to continue to build our understanding of the role of MeCP2 on neuronal and glial structure and function, its greater impact on neural development, and potential new therapeutic avenues.
Identifiants
pubmed: 30502397
pii: S1074-7427(18)30262-4
doi: 10.1016/j.nlm.2018.11.007
pmc: PMC8040058
mid: NIHMS1061315
pii:
doi:
Substances chimiques
Mecp2 protein, mouse
0
Methyl-CpG-Binding Protein 2
0
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
106962Subventions
Organisme : NICHD NIH HHS
ID : U54 HD079123
Pays : United States
Organisme : NINDS NIH HHS
ID : R21 NS100085
Pays : United States
Organisme : NINDS NIH HHS
ID : F32 NS010085
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS113140
Pays : United States
Organisme : NCRR NIH HHS
ID : S10 RR027445
Pays : United States
Informations de copyright
Copyright © 2018 Elsevier Inc. All rights reserved.
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