title: Activated remodeling and N-methyl-D-aspartate (NMDA) receptors in cortical dysplasia.
abstract: Cortical dysplasia is now recognized as one of the major etiologies causing intractable epilepsy in childhood. Dysplastic cortex displays cortical dyslamination, which is often associated with dysmorphic large neurons and less frequently with balloon cells. The dysmorphic large neurons are commonly located in the subcortical white matter and cerebral cortex, with enlarged nuclei with a single prominent nucleolus and showing aberrant cytoskeletal changes. I have shown that dysmorphic large neurons have several immature types of cytoskeletal proteins, such as the low-molecular-weight form of microtubule-associated protein 2 (MAP2) and MAP1B, which are involved in the outgrowth and modeling of neuronal processes in the immature brain. I have also reported that dysmorphic large neurons also have enhanced gene expression of growth-associated protein GAP43, which is a phosphoprotein enriched at presynaptic nerve terminals and is thought to be involved in axonal outgrowth and plasticity in synaptic connections. Finally, I have shown that the N-methyl-D-aspartate acid (NMDA) receptor R1 gene is up-regulated in the dysmorphic large neurons and nearly normal-sized neurons located in the dysplastic cortex. This evidence suggests that growth of neuronal processes and activated excitatory synaptic remodeling exist in the epileptic conditions of cortical dysplasia.
authors.affiliation: Department of Pediatrics, Dokkyo University School of Medicine, Tochigi, Japan. yhideo@dokkyomed.ac.jp
authors.forename: Hideo
authors.initials: H
authors.lastname: Yamanouchi
mesh_terms: D002540:Cerebral Cortex / Q000002:abnormalities*; D002648:Child; D004827:Epilepsy / Q000209:etiology*; D019922:GAP-43 Protein / Q000502:physiology; D006801:Humans; D009473:Neuronal Plasticity / Q000502:physiology; D009474:Neurons / Q000502:physiology; D038081:Organogenesis / Q000502:physiology*; D016194:Receptors, N-Methyl-D-Aspartate / Q000502:physiology
chemical_list: D019922:GAP-43 Protein; D016194:Receptors, N-Methyl-D-Aspartate