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Loss of Satb2 in the Cortex and Hippocampus Leads to Abnormal Behaviors in Mice

Satb2-associated syndrome (SAS) is a genetic disorder that results from the deletion or mutation of one allele within the Satb2 locus. Patients with SAS show behavioral abnormalities, including developmental delay/intellectual disability, hyperactivity, and symptoms of autism. To address the role of...

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Autores principales: Zhang, Qiong, Huang, Ying, Zhang, Lei, Ding, Yu-Qiang, Song, Ning-Ning
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6380165/
https://www.ncbi.nlm.nih.gov/pubmed/30809123
http://dx.doi.org/10.3389/fnmol.2019.00033
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author Zhang, Qiong
Huang, Ying
Zhang, Lei
Ding, Yu-Qiang
Song, Ning-Ning
author_facet Zhang, Qiong
Huang, Ying
Zhang, Lei
Ding, Yu-Qiang
Song, Ning-Ning
author_sort Zhang, Qiong
collection PubMed
description Satb2-associated syndrome (SAS) is a genetic disorder that results from the deletion or mutation of one allele within the Satb2 locus. Patients with SAS show behavioral abnormalities, including developmental delay/intellectual disability, hyperactivity, and symptoms of autism. To address the role of Satb2 in SAS-related behaviors and generate an SAS mouse model, Satb2 was deleted in the cortex and hippocampus of Emx1-Cre; Satb2(flox/flox) [Satb2 conditional knockout (CKO)] mice. Satb2 CKO mice showed hyperactivity, increased impulsivity, abnormal social novelty, and impaired spatial learning and memory. Furthermore, we also found that the development of neurons in cortical layer IV was defective in Satb2 CKO mice, as shown by the loss of layer-specific gene expression and abnormal thalamocortical projections. In summary, the abnormal behaviors revealed in Satb2 CKO mice may reflect the SAS symptoms associated with Satb2 mutation in human patients, possibly due to defective development of cortical neurons in multiple layers including alterations of their inputs/outputs.
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spelling pubmed-63801652019-02-26 Loss of Satb2 in the Cortex and Hippocampus Leads to Abnormal Behaviors in Mice Zhang, Qiong Huang, Ying Zhang, Lei Ding, Yu-Qiang Song, Ning-Ning Front Mol Neurosci Neuroscience Satb2-associated syndrome (SAS) is a genetic disorder that results from the deletion or mutation of one allele within the Satb2 locus. Patients with SAS show behavioral abnormalities, including developmental delay/intellectual disability, hyperactivity, and symptoms of autism. To address the role of Satb2 in SAS-related behaviors and generate an SAS mouse model, Satb2 was deleted in the cortex and hippocampus of Emx1-Cre; Satb2(flox/flox) [Satb2 conditional knockout (CKO)] mice. Satb2 CKO mice showed hyperactivity, increased impulsivity, abnormal social novelty, and impaired spatial learning and memory. Furthermore, we also found that the development of neurons in cortical layer IV was defective in Satb2 CKO mice, as shown by the loss of layer-specific gene expression and abnormal thalamocortical projections. In summary, the abnormal behaviors revealed in Satb2 CKO mice may reflect the SAS symptoms associated with Satb2 mutation in human patients, possibly due to defective development of cortical neurons in multiple layers including alterations of their inputs/outputs. Frontiers Media S.A. 2019-02-12 /pmc/articles/PMC6380165/ /pubmed/30809123 http://dx.doi.org/10.3389/fnmol.2019.00033 Text en Copyright © 2019 Zhang, Huang, Zhang, Ding and Song. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neuroscience
Zhang, Qiong
Huang, Ying
Zhang, Lei
Ding, Yu-Qiang
Song, Ning-Ning
Loss of Satb2 in the Cortex and Hippocampus Leads to Abnormal Behaviors in Mice
title Loss of Satb2 in the Cortex and Hippocampus Leads to Abnormal Behaviors in Mice
title_full Loss of Satb2 in the Cortex and Hippocampus Leads to Abnormal Behaviors in Mice
title_fullStr Loss of Satb2 in the Cortex and Hippocampus Leads to Abnormal Behaviors in Mice
title_full_unstemmed Loss of Satb2 in the Cortex and Hippocampus Leads to Abnormal Behaviors in Mice
title_short Loss of Satb2 in the Cortex and Hippocampus Leads to Abnormal Behaviors in Mice
title_sort loss of satb2 in the cortex and hippocampus leads to abnormal behaviors in mice
topic Neuroscience
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6380165/
https://www.ncbi.nlm.nih.gov/pubmed/30809123
http://dx.doi.org/10.3389/fnmol.2019.00033
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