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Neuronal Migration and AUTS2 Syndrome

Department of Biochemistry and Cellular Biology, National Institute of Neuroscience, NCNP, Tokyo 187-8502, Japan
Author to whom correspondence should be addressed.
Academic Editor: Kazuhito Toyooka
Brain Sci. 2017, 7(5), 54;
Received: 19 April 2017 / Revised: 8 May 2017 / Accepted: 11 May 2017 / Published: 14 May 2017
(This article belongs to the Special Issue Neuronal Migration and Cortical Development)
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Neuronal migration is one of the pivotal steps to form a functional brain, and disorganization of this process is believed to underlie the pathology of psychiatric disorders including schizophrenia, autism spectrum disorders (ASD) and epilepsy. However, it is not clear how abnormal neuronal migration causes mental dysfunction. Recently, a key gene for various psychiatric diseases, the Autism susceptibility candidate 2 (AUTS2), has been shown to regulate neuronal migration, which gives new insight into understanding this question. Interestingly, the AUTS2 protein has dual functions: Cytoplasmic AUTS2 regulates actin cytoskeleton to control neuronal migration and neurite extension, while nuclear AUTS2 controls transcription of various genes as a component of the polycomb complex 1 (PRC1). In this review, we discuss AUTS2 from the viewpoint of human genetics, molecular function, brain development, and behavior in animal models, focusing on its role in neuronal migration. View Full-Text
Keywords: Autism; intellectual disabilities; AUTS2 syndrome; Rac1; neuritogenesis; neuronal migration; cytoskeleton; PRC1 Autism; intellectual disabilities; AUTS2 syndrome; Rac1; neuritogenesis; neuronal migration; cytoskeleton; PRC1

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Hori, K.; Hoshino, M. Neuronal Migration and AUTS2 Syndrome. Brain Sci. 2017, 7, 54.

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