Abnormal social behaviors and altered gene expression rates in a mouse model for Potocki-Lupski syndrome.

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Serval ID
serval:BIB_FA3FDDAE1B36
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Abnormal social behaviors and altered gene expression rates in a mouse model for Potocki-Lupski syndrome.
Journal
Human Molecular Genetics
Author(s)
Molina J., Carmona-Mora P., Chrast J., Krall P.M., Canales C.P., Lupski J.R., Reymond A., Walz K.
ISSN
1460-2083[electronic]
Publication state
Published
Issued date
2008
Peer-reviewed
Oui
Volume
17
Number
16
Pages
2486-2495
Language
english
Abstract
The Potocki-Lupski syndrome (PTLS) is associated with a microduplication of 17p11.2. Clinical features include multiple congenital and neurobehavioral abnormalities and autistic features. We have generated a PTLS mouse model, Dp(11)17/+, that recapitulates some of the physical and neurobehavioral phenotypes present in patients. Here, we investigated the social behavior and gene expression pattern of this mouse model in a pure C57BL/6-Tyr(c-Brd) genetic background. Dp(11)17/+ male mice displayed normal home-cage behavior but increased anxiety and increased dominant behavior in specific tests. A subtle impairment in the preference for a social target versus an inanimate target and abnormal preference for social novelty (the preference to explore an unfamiliar mouse versus a familiar one) was also observed. Our results indicate that these animals could provide a valuable model to identify the specific gene(s) that confer abnormal social behaviors and that map within this delimited genomic deletion interval. In a first attempt to identify candidate genes and for elucidating the mechanisms of regulation of these important phenotypes, we directly assessed the relative transcription of genes within and around this genomic interval. In this mouse model, we found that candidates genes include not only most of the duplicated genes, but also normal-copy genes that flank the engineered interval; both categories of genes showed altered expression levels in the hippocampus of Dp(11)17/+ mice.
Keywords
Animals, Autistic Disorder/genetics, Autistic Disorder/physiopathology, Behavior, Animal, Brain/growth & development, Disease Models, Animal, Female, Gene Expression, Gene Expression Profiling, Humans, Male, Mice, Mice, Inbred C57BL, Organ Size, Phenotype, Species Specificity
Pubmed
Web of science
Open Access
Yes
Create date
18/06/2009 13:45
Last modification date
25/09/2019 7:11
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