Preferential recruitment of ataxin-3 independent of expanded polyglutamine: an immunohistochemical study on Marinesco bodies
Preferential recruitment of ataxin-3 independent of expanded polyglutamine: an immunohistochemical study on Marinesco bodies
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不依赖于扩展的聚谷氨酰胺的 ataxin-3 的优先招募：Marinesco 小体的免疫组织化学研究
DOI：
10.1136/jnnp.71.4.518
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发表时间：
2001
期刊：
影响因子：
--
通讯作者：
H. Mizusawa
中科院分区：
文献类型：
--
作者：
H. Fujigasaki;T. Uchihara;J. Takahashi;H. Matsushita;A. Nakamura;S. Koyano;K. Iwabuchi;S. Hirai;H. Mizusawa
在Marinesco小体的免疫组织化学研究中，一种常见于肝性脑病患者黑质神经元的神经元核内包涵体显示，其中一种多聚谷氨酰胺蛋白，共济失调蛋白-3，优先被募集到该包涵体中，而其他多聚谷氨酰胺蛋白（共济失调蛋白-2和TATA盒结合蛋白）则没有。这表明，募集的每一个聚谷氨酰胺蛋白可能受到不同的调节。因为这种核包涵体被认为是在细胞应激反应中形成的，如在肝性脑病中发生的，即使在没有扩增的CAG/聚谷氨酰胺重复序列的情况下，共济失调蛋白-3和泛素募集到Marinesco小体中可能代表细胞对与扩增的CAG/聚谷氨酰胺无关的有害外部刺激的反应。
In an immunohistochemical study of Marinesco bodies—a neuronal intranuclear inclusion often seen in neurons of the substantia nigra of patients with hepatic encephalopathy—it was shown that one of the polyglutamine proteins, ataxin-3, is preferentially recruited into this inclusion, whereas other polyglutamine proteins (ataxin-2 and TATA box-binding protein) are not. This suggests that recruitment of each of the polyglutamine proteins may be differently regulated. Because this nuclear inclusion is thought to be formed in response to cellular stress, as occurs in hepatic encephalopathy, even in the absence of an expanded CAG/polyglutamine repeat, recruitment of ataxin-3 and ubiquitin into Marinesco bodies may represent a cellular response to noxious external stimuli unrelated to expanded CAG/polyglutamine.