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Comparative Study
. 2009 Sep 8;73(10):746-53.
doi: 10.1212/WNL.0b013e3181af3397. Epub 2009 Jun 17.

Layer-specific genes reveal a rudimentary laminar pattern in human nodular heterotopia

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Comparative Study

Layer-specific genes reveal a rudimentary laminar pattern in human nodular heterotopia

R Garbelli et al. Neurology. .

Abstract

Objective: To define distinctive features of nodular heterotopia in specimens derived from drug-resistant patients with epilepsy by evaluating mRNA expression of three different layer-specific markers: Rorbeta, Er81, and Nurr1.

Methods: We analyzed the expression profile of these genes, recognized as markers mainly expressed in layer IV for Rorbeta, in layer V for Er81, and in layer VI for Nurr1, in surgical samples from 14 epileptic patients, using in situ hybridization. Six patients had subcortical nodular heterotopia and 8 patients were controls. The intrinsic organization of nodular formations and of the overlaying neocortex was assessed.

Results: In all patients, the 3 selected genes showed high cortical laminar specificity. In subcortical nodular heterotopia, the different gene expression profiles revealed a rudimentary laminar organization of the nodules. In the overlaying cortex, fewer cells expressed the 3 genes in the appropriate specific layer as compared to controls.

Conclusions: These data provide new insights into possible ontogenetic mechanisms of nodular heterotopia formation and show the potential role of layer-specific markers to elucidate the neuropathology of malformations of cortical development.

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Comment in

  • Nodular heterotopia is built upon layers.
    Ferland RJ, Guerrini R. Ferland RJ, et al. Neurology. 2009 Sep 8;73(10):742-3. doi: 10.1212/WNL.0b013e3181b529b1. Epub 2009 Jul 22. Neurology. 2009. PMID: 19625704 No abstract available.

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