NEURONAL CIRCUIT FORMATION IN THE GENETICAL AND EXPERIMENTAL BRAIN MALFORMATION ANIMAL.

遗传性多指小鼠品系纯合子表现出大脑某一特定区域皮质层层破坏和S-100β免疫反应元件显著减少。为了在细胞水平上了解皮质的异常形成，用溴脱氧尿苷、S-100β和胶质纤维酸性蛋白免疫组织化学方法研究了皮质神经元的迁移和胶质细胞的发育。纯合子小鼠(PDN/PDN)表现出不同的异常模式。新生PDN小鼠尾侧背侧皮质可见不规则的GFAP阳性放射状胶质细胞和神经元迁移障碍。S-100β阳性细胞在该区域减少。提示皮质分层异常与神经元迁移障碍和胶质细胞异常密切相关，尤其与S-100β免疫反应细胞的显著减少密切相关。

Homozygote of a mouse strain with genetic polydactyly (Pdn) show disrupted cortical lamination and significant decrease of S-100beta immunoreactive elements in a particular area of the brain. In order to understand the abnormal cortical formation at the cellular level, migration of cortical neurons and development of glia cells were studied using bromodeoxyuridine (BrdU), S-100beta and glial fibrillary acidic protein (GFAP) immunohistochemistry. Homozygous mice (Pdn/Pdn) displayd a variable pattern of abnormalities. Irregular GFAP-positive radial glia cells and disturbance of neuronal migration were found in a circumscribed area of the caudo-dorsal cortex of newborn Pdn mouse. The number of S-100beta-positive cells was reduced in this area. The present results suggest that abnormal cortical lamination closely correlates with disturbance of neuronal migration and abnormalities of glia cells, especially a significant decrease of S-100beta immunoreactive cells.