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Dendritic spine pathology in autism: lessons learned from mouse models |
| Qiangge Zhang, Dingxi Zhou, Guoping Feng |
| 1. McGovern Institute for Brain Research, Department of Brain and Cognitive Sciences, Massachusetts Institute of Technology, Cambridge, MA 02139, USA.
2. Stanley Center for Psychiatric Research, Broad Institute of MIT and Harvard, Cambridge, MA 02142, USA
3. School of Life Sciences, Peking University, Beijing 100871, China |
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Abstract Abstract Autism spectrum disorders (ASD) area group of neurodevelopmental disorders that affect
up to 1.5% of population in the world. Recent largescale genomic studies show that genetic causes of ASD are very heterogeneous. Gene ontology, pathwayanalysis and animal model studies have revealed several potential converging mechanisms including postsynaptic dysfunction of excitatory synapses. In this review, we focus on the structural and functional specializations
of dendritic spines, and describe their defects in ASD.We use Fragile X syndrome, Rett syndrome and Phelan-McDermid syndrome, three of the most studied neurodevelopmental disorders with autism features, as examples to demonstrate the significant contribution made by mouse models towards the understanding of monogenic ASD. We envision that the development and application of new technologies to study the function of dendritic spines in valid animal models will eventually
lead to innovative treatments for ASD.
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| Keywords
synapse, dendritic spine, autism,FMR1, MECP2, SHANK3, animal model
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Issue Date: 18 December 2016
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