慢性炎症性脱髓鞘性多发性神经病：claudin-5减少和紧密连接蛋白1重新定位。
Chronic inflammatory demyelinating polyneuropathy: decreased claudin-5 and relocated ZO-1.
作者信息
Kanda T, Numata Y, Mizusawa H
机构信息
Department of Neurology and Neurological Science, Tokyo Medical and Dental University Graduate School, 1-5-45 Yushima, Bunkyo-ku, Tokyo 113-8519, Japan.
出版信息
J Neurol Neurosurg Psychiatry. 2004 May;75(5):765-9. doi: 10.1136/jnnp.2003.025692.
OBJECTIVES
To clarify the dynamics of molecules composing the blood-nerve barrier (BNB) in inflammatory neuropathies.
METHODS
The expression of four tight junction (TJ) proteins-claudin-1, claudin-5, occludin, and ZO-1-was analysed immunohistochemically in sural nerve biopsy specimens obtained from patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).
RESULTS
Claudin-1 was detected only in perineurial cells, whereas claudin-5 was present in endothelial cells, irrespective of vessel location or size. Occludin and ZO-1 were found in perineurial cells, in addition to some epineurial and endoneurial endothelial cells. In CIDP, percentages of endoneurial small vessels immunoreactive for claudin-5 were significantly decreased, as were ZO-1 immunoreactive endoneurial small vessels, with staining localised to interfaces between cells. Claudin-1 and occludin immunoreactivity did not differ appreciably between the neuropathies examined.
CONCLUSIONS
The downregulation of claudin-5 and altered localisation of ZO-1 seen in CIDP specimens may indicate that BNB derangement occurs in inflammatory neuropathies. Further investigation of TJ molecules may suggest new treatments based on properties of the BNB.
目的
阐明炎性神经病中构成血-神经屏障（BNB）的分子动态变化。
方法
采用免疫组织化学方法分析慢性炎性脱髓鞘性多发性神经根神经病（CIDP）患者腓肠神经活检标本中四种紧密连接（TJ）蛋白——闭合蛋白-1、闭合蛋白-5、封闭蛋白和紧密连接蛋白1（ZO-1）的表达情况。
结果
闭合蛋白-1仅在神经束膜细胞中检测到，而闭合蛋白-5存在于内皮细胞中，与血管位置或大小无关。除了一些神经外膜和神经内膜内皮细胞外，在神经束膜细胞中也发现了封闭蛋白和紧密连接蛋白1。在CIDP中，闭合蛋白-5免疫反应阳性的神经内膜小血管百分比显著降低，紧密连接蛋白1免疫反应阳性的神经内膜小血管百分比也显著降低，染色定位于细胞间界面。在所检查的神经病中，闭合蛋白-1和封闭蛋白的免疫反应性没有明显差异。
结论
CIDP标本中闭合蛋白-5的下调和紧密连接蛋白1定位的改变可能表明炎性神经病中存在BNB紊乱。对TJ分子的进一步研究可能会基于BNB的特性提出新的治疗方法。
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