Neuronal migration disorders in humans and in mouse models - an overview

被引:43
作者
Copp, AJ
Harding, BN
机构
[1] UCL, Neural Dev Unit, Inst Child Hlth, London WC1N 1EH, England
[2] Great Ormond St Hosp Sick Children, Dept Histopathol, London WC1N 1JH, England
基金
英国惠康基金;
关键词
neuronal migration disorders; epilepsy; lissencephaly; neuroblasts; mutant genes; reelin;
D O I
10.1016/S0920-1211(99)00047-9
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
The spectrum of neuronal migration disorders (NMD) in humans encompasses developmental brain defects with a range of clinical and pathological features. A simple classification distinguishes agyria/pachygyria, heterotopia, polymicrogyria and cortical dysplasia as distinct clinico-pathological entities. Many of these conditions are associated with intractable epilepsy. When considering the pathogenesis of NMD, a critical developmental process is the migration of neuroblasts along the processes of radial glia during the formation of the layered structure of the cerebral cortex. In addition, faulty cytodifferentiation and programmed cell death play important roles in the generation of dysplasias and heterotopias respectively. A number of genes have been identified that participate in the regulation of neuronal migration. Mouse models, in which these genes are mutated, provide insight into the developmental pathways that underlie normal and abnormal neuronal migration. (C) 1999 Elsevier Science B.V. All rights reserved.
引用
收藏
页码:133 / 141
页数:9
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