Distinct Defects in Spine Formation or Pruning in Two Gene Duplication Mouse Models of Autism

doi:10.1007/s12264-017-0111-8

	Distinct Defects in Spine Formation or Pruning in Two Gene Duplication Mouse Models of Autism
	Wang, Miao 1,2; Li, Huiping 3; Takumi, Toru 4; Qiu, Zilong 1; Xu, Xiu 3; Yu, Xiang1,2,5 ; Bian, Wen-Jie 1,2
	2017-04
发表期刊	NEUROSCIENCE BULLETIN
ISSN	1673-7067
卷号	33 期号:2 页码:143-152
发表状态	已发表
DOI	10.1007/s12264-017-0111-8
摘要	Autism spectrum disorder (ASD) encompasses a complex set of developmental neurological disorders, characterized by deficits in social communication and excessive repetitive behaviors. In recent years, ASD is increasingly being considered as a disease of the synapse. One main type of genetic aberration leading to ASD is gene duplication, and several mouse models have been generated mimicking these mutations. Here, we studied the effects of MECP2 duplication and human chromosome 15q11-13 duplication on synaptic development and neural circuit wiring in the mouse sensory cortices. We showed that mice carrying MECP2 duplication had specific defects in spine pruning, while the 15q11-13 duplication mouse model had impaired spine formation. Our results demonstrate that spine pathology varies significantly between autism models and that distinct aspects of neural circuit development may be targeted in different ASD mutations. Our results further underscore the importance of gene dosage in normal development and function of the brain.
关键词	Autism Autism spectrum disorder Spine Spine formation Spinogenesis Spine pruning Gene duplication MECP2 15q11-13 duplication
收录类别	SCI
语种	英语
资助项目	Science and Technology Commission of Shanghai Municipality, China[16XD1404800]
WOS研究方向	Neurosciences & Neurology
WOS类目	Neurosciences
WOS记录号	WOS:000398725200003
出版者	SPRINGER
WOS关键词	COPY-NUMBER VARIATION ; DOWN-SYNDROME ; DENDRITIC SPINES ; SPECTRUM DISORDERS ; CEREBRAL-CORTEX ; FRAGILE-X ; BEHAVIORAL ABNORMALITIES ; DIVERSE REGIONS ; RETT-SYNDROME ; MECP2
原始文献类型	Article
引用统计
文献类型	期刊论文
条目标识符	https://kms.shanghaitech.edu.cn/handle/2MSLDSTB/1444
专题	生命科学与技术学院_特聘教授组_于翔组
通讯作者	Xu, Xiu; Yu, Xiang; Bian, Wen-Jie
作者单位	1.Chinese Acad Sci, Inst Neurosci, State Key Lab Neurosci, CAS Ctr Excellence Brain Sci & Intelligence Techn, Shanghai 200031, Peoples R China 2.Univ Chinese Acad Sci, Beijing 100049, Peoples R China 3.Fudan Univ, Dept Child Healthcare, Childrens Hosp, Shanghai 201102, Peoples R China 4.RIKEN Brain Sci Inst, Wako, Saitama 3510198, Japan 5.ShanghaiTech Univ, Sch Life Sci & Technol, Shanghai 201210, Peoples R China
通讯作者单位	生命科学与技术学院
推荐引用方式 GB/T 7714	Wang, Miao,Li, Huiping,Takumi, Toru,et al. Distinct Defects in Spine Formation or Pruning in Two Gene Duplication Mouse Models of Autism[J]. NEUROSCIENCE BULLETIN,2017,33(2):143-152.
APA	Wang, Miao.,Li, Huiping.,Takumi, Toru.,Qiu, Zilong.,Xu, Xiu.,...&Bian, Wen-Jie.(2017).Distinct Defects in Spine Formation or Pruning in Two Gene Duplication Mouse Models of Autism.NEUROSCIENCE BULLETIN,33(2),143-152.
MLA	Wang, Miao,et al."Distinct Defects in Spine Formation or Pruning in Two Gene Duplication Mouse Models of Autism".NEUROSCIENCE BULLETIN 33.2(2017):143-152.