Expression of NF2 gene product merlin in arachnoid villi and meningiomas.

K Sakuda, Y Kohda, T Matsumoto, C Park, A Seto, Y Tohma, M Hasegawa, S Kida, H Nitta, T Yamashima, J Yamashita
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Abstract

Neurofibromatosis type 2 (NF2) gene encodes a novel 595 amino acid protein named merlin. Recently, Ruttledge et al demonstrated inactivation of NF2 gene in approximately 60% of sporadically occurring meningiomas. Merlin is thought to physiologically exist beneath the cell membrane, and to form a part of modulation in signal transduction, for example, information concerning contact inhibition. In NF2-related tumors, it is supposed that the mutation of merlin results in loss of this signal transduction leading to tumorigenesis. In this paper, we investigated the expression of NF2 gene product merlin in arachnoid villi and meningiomas. The immunohistochemical staining of merlin showed a striking contrast between arachnoid villi and meningiomas. In arachnoid cells, merlin was labeled in the whole cytoplasm, but not within the nuclei. In contrast, in meningiomas, immunoreactivity of merlin was mainly seen in the nuclei. These results suggest that arachnoid cells with normal merlin are capable of normal signal transduction, whereas meningioma cells with mutated merlin show impairment of signal transduction which may lead to tumorigenesis.

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NF2基因产物merlin在蛛网膜绒毛和脑膜瘤中的表达。
2型神经纤维瘤病(NF2)基因编码一种新的595个氨基酸的蛋白质,名为merlin。最近,Ruttledge等人证实,约60%的散发性脑膜瘤存在NF2基因失活。Merlin被认为在生理上存在于细胞膜之下,并在信号转导中形成调制的一部分,例如,有关接触抑制的信息。在nf2相关的肿瘤中,人们认为merlin的突变导致这种信号转导的丧失,从而导致肿瘤发生。本文研究了NF2基因产物merlin在蛛网膜绒毛和脑膜瘤中的表达。免疫组化染色显示蛛网膜绒毛和脑膜瘤有明显的差异。在蛛网膜细胞中,merlin在整个细胞质中被标记,但在细胞核中不被标记。相反,在脑膜瘤中,merlin的免疫反应性主要见于细胞核。这些结果表明,具有正常梅林的蛛网膜细胞具有正常的信号转导能力,而具有突变梅林的脑膜瘤细胞表现出信号转导障碍,可能导致肿瘤发生。
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