Plectin in the human central nervous system:: predominant expression at pia/glia and endothelia/glia interfaces
Plectin in the human central nervous system:: predominant expression at pia/glia and endothelia/glia interfaces
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DOI：
10.1007/s004010050885
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发表时间：
1998-09-01
影响因子：
12.7
通讯作者：
Elger, CE
中科院分区：
文献类型：
--
作者：
Lie, AA;Schröder, R;Elger, CE
凝集素是一种高分子量蛋白质，作为一种通用的细胞骨架交联分子。最近已确定人类plectin基因的突变导致常染色体隐性遗传疾病单纯性大疱性表皮病伴肌营养不良症（EBS-MD）。EBS-MD患者的亚组显示神经退行性疾病的迹象，表明有缺陷的网蛋白的表达也可能干扰人类中枢神经系统的结构和功能完整性。然而，在人脑中的表达模式仍然是未知的。因此，我们分析了免疫组化分布的plectin在正常海马标本在尸检中，并在新皮层和海马组织的癫痫手术患者。在一般情况下，果胶免疫反应细胞被确定为毛细血管内皮细胞和星形胶质细胞。在所有标本中观察到的一个显著特征是星形胶质细胞端足邻接血管和软脑膜表面的突出的网蛋白免疫反应性。此外，对患有阿蒙角硬化症（AHS）的癫痫患者的海马组织的分析揭示了反应性星形胶质细胞的强烈的网素标记。后者的发现表明，上调的plectin，这平行的胶质细胞酸性蛋白的增加，可能是反应性星形胶质细胞的一般特征。在人大脑中的软脑膜/神经胶质和内皮细胞/神经胶质界面处主要表达的plectin表明，plectin可能在血脑屏障和软脑膜的结构组织中具有不可或缺的作用。
Plectin is a high molecular weight protein that serves as a versatile cytoskeletal cross-linker molecule. Mutations of the human plectin gene have recently been identified to cause the autosomal recessive disorder epidermolysis bullosa simplex with muscular dystrophy (EBS-MD). A subgroup of EBS-MD patients display signs of a neurodegenerative disorder suggesting that the expression of defective plectin may also interfere with the structural and functional integrity of the human central nervous system. However, the expression pattern of plectin in the human brain is still unknown. We therefore analyzed the immunohistochemical distribution of plectin in normal hippocampal specimens obtained at autopsy and in neocortical and hippocampal tissue of patients who had undergone epilepsy surgery. In general, plectin-immunoreactive cells were identified as capillary endothelia and astrocytes. A striking feature seen in all specimens was the accentuated plectin immunoreactivity of astrocytic end feet abutting on blood vessels and on the pial surface. Furthermore, the analysis of hippocampal tissue of epilepsy patients with Ammon's horn sclerosis (AHS) revealed a strong plectin labeling of reactive astrocytes. The latter finding suggests that the up-regulation of plectin, which parallels the increase of glial fibrillary acidic protein, may be a general feature of reactive astroglia. The predominant expression of plectin at pia/glia and endothelia/glia interfaces in the human brain indicates that plectin may have an integral role in the structural organization of the blood-brain barrier and the leptomeninges.