Distribution of the epilepsy-related Lgi1 protein in rat cortical neurons
Distribution of the epilepsy-related Lgi1 protein in rat cortical neurons
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DOI：
10.1007/s00418-009-0637-6
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发表时间：
2009-11-01
影响因子：
2.3
通讯作者：
Nobile, Carlo
中科院分区：
文献类型：
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作者：
Malatesta, Manuela;Furlan, Sandra;Nobile, Carlo
Lgi1蛋白参与了常染色体显性遗传性外侧颞叶癫痫的发病机制，因为在受试者和家系中已经发现了富含亮氨酸的胶质瘤灭活1(LGI1)基因的突变；然而，Lgi1的功能仍不清楚。定义正常皮质组织中Lgi1在细胞内的精细分布将有助于理解其作用并设想致病机制。因此，我们结合生化、免疫组织化学和免疫电子显微镜对正常大鼠大脑皮质中的Lgi1进行了分析。我们的结果表明，Lgi1是一种胞质蛋白，分布在胞体和神经元的突起中：它位于合成的粗面内质网，在高尔基复合体中进行糖基化，靠近神经管和神经丝，特别是在轴突中，但在突触和神经膜中很少。Lgi1与轴突细胞骨架结构的相关性表明，Lgi1可能通过马达蛋白沿轴突运输，或作为轴突流量的载体，也可能参与细胞骨架组织的调节。
The Lgi1 protein is involved in the pathogenesis of autosomal dominant lateral temporal epilepsy because mutations in the leucine-rich, glioma-inactivated 1 (LGI1) gene have been found in affected subjects and families; however, the function of Lgi1 is still unclear. Definition of the fine intracellular distribution of Lgi1 in normal cortical tissue would add in understanding its role and envisage pathogenetic mechanisms. Therefore, we analyzed normal rat cortex for Lgi1 by combining biochemistry, immunohistochemistry and immunoelectron microscopy. Our results demonstrate that Lgi1 is a cytoplasmic protein distributed both in the soma and in the processes of neurons: it occurs on the rough endoplasmic reticulum, the site of synthesis, in the Golgi complex, where it undergoes glycosylation, and in close proximity to neurotubules and neurofilaments, especially in the axons, but it is scarce at synapses and the neurilemma. Lgi1 association with axonal cytoskeletal structures would imply Lgi1 is either transported along axons by motor proteins, or playing some role as a carrier in the axonal flux, or it could be involved in the regulation of cytoskeletal organization.