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

Neuronal migration disorders in humans and in mouse models—an overview 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. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Epilepsy Research Elsevier

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

Epilepsy Research, Volume 36 (2) – Sep 1, 1999

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Publisher
Elsevier
Copyright
Copyright © 1999 Elsevier Science B.V.
ISSN
0920-1211
D.O.I.
10.1016/S0920-1211(99)00047-9
Publisher site
See Article on Publisher Site

Abstract

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.

Journal

Epilepsy ResearchElsevier

Published: Sep 1, 1999

References

  • The subplate, a transient neocortical structure: Its role in the development of connections between thalamus and cortex
    Allendoerfer, K.L.; Shatz, C.J.
  • Widespread neuronal ectopia associated with secondary defects in cerebrocortical chondroitin sulfate proteoglycans and basal lamina in MARCKS-deficient mice
    Blackshear, P.J.; Silver, J.; Nairn, A.C.; Sulik, K.K.; Squier, M.V.; Stumpo, D.J.; Tuttle, J.S.
  • The fates of cells in the developing cerebral cortex of normal and methylazoxymethanol acetate-lesioned mice
    Gillies, K.; Price, D.J.
  • Altered forebrain and hindbrain development in mice mutant for the Gsh- 2 homeobox gene
    Szucsik, J.C.; Witte, D.P.; Li, H.; Pixley, S.K.; Small, K.M.; Potter, S.S.

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