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小脑神经元迁移障碍中的星形胶质细胞和小胶质细胞。

Astroglia and microglia in cerebellar neuronal migration disturbances.

作者信息

Laure-Kamionowska Milena, Maślińska Danuta

机构信息

Department of Experimental and Clinical Neuropathology, Medical Research Centre, Polish Academy of Sciences, Pawiñskiego 5, 02-106 Warszawa, Poland.

出版信息

Folia Neuropathol. 2007;45(4):205-12.

Abstract

Between the neuronal and glial cells there is a close relationship conditioning a tight morphological correlation and proper functional interplay. Disturbed interaction between glial and neuronal components leads to inappropriate neural circuits. The reflection of the failure of neural circuit organisation is the picture of morphological changes of neurons and glia. The appearance of microglia and astroglia was analysed in a defectively formed cellular network due to cerebellar neuronal migration disturbances. Focal disruption of neuron migration leads to their differentiation in an abnormal position manifested as heterotopias and cortical anomalies. Neurons that had lost their proper migratory way and heterotopically settled in the white matter were encircled by GFAP-positive astrocytes, with morphology appropriate for surrounding white matter. The microglial cells infiltrated the parenchyma within the heterotopic neurons playing a role in their elimination. In the cerebellar cortical malformations astrocytes were grouped near the Purkinje cells. In the minimal cortical dysplasia the increased number of astrocytes supported the neurons. Impaired morphological components of the glial-pial barrier were observed. In the massive cortical malformations a few degenerated astrocytes followed the disarranged Purkinje cells, while microglia and Bergmann glia fibres were not present. Absence of cells supporting and organizing the cerebellar cortex had an effect on loss of Purkinje cell shape, their disorientation and abnormal position. The appearance and localisation of the astroglia and microglia in the abnormal cerebellar circuitry due to migration disturbances is dependent on the pathomechanism of the anomalies.

摘要

神经元细胞和神经胶质细胞之间存在着密切的关系,这种关系决定了紧密的形态学关联和恰当的功能相互作用。神经胶质细胞和神经元成分之间的相互作用紊乱会导致神经回路异常。神经回路组织失败的表现是神经元和神经胶质细胞形态变化的情况。在由于小脑神经元迁移障碍导致的细胞网络形成缺陷中,分析了小胶质细胞和星形胶质细胞的出现情况。神经元迁移的局部破坏导致它们在异常位置分化,表现为异位和皮质异常。失去正常迁移路径并异位定居在白质中的神经元被GFAP阳性星形胶质细胞包围,其形态适合于周围白质。小胶质细胞浸润到异位神经元内的实质中,在其清除过程中发挥作用。在小脑皮质畸形中,星形胶质细胞聚集在浦肯野细胞附近。在最小型皮质发育不良中,星形胶质细胞数量增加对神经元起到了支持作用。观察到神经胶质-软脑膜屏障的形态成分受损。在大规模皮质畸形中,一些退化的星形胶质细胞跟随排列紊乱的浦肯野细胞,而小胶质细胞和伯格曼胶质纤维不存在。缺乏支持和组织小脑皮质的细胞会导致浦肯野细胞形状丧失、定向障碍和位置异常。由于迁移障碍导致的异常小脑回路中星形胶质细胞和小胶质细胞的出现和定位取决于异常的发病机制。

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