General transcriptional repression by polyglutamine disease proteins is not directly linked to the presence of inclusion bodies
General transcriptional repression by polyglutamine disease proteins is not directly linked to the presence of inclusion bodies
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DOI：
10.1016/j.bbrc.2003.11.086
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发表时间：
2004-01-02
影响因子：
3.1
通讯作者：
Okazawa, H
中科院分区：
文献类型：
--
作者：
Hoshino, M;Tagawa, K;Okazawa, H
通过使用直接免疫细胞化学的BrU纳入RNA的核，我们评估了突变亨廷顿蛋白和共济失调蛋白-1对一般的转录在初级皮质和小脑神经元的影响。我们的定量分析清楚地表明，这些突变的多聚谷氨酰胺疾病蛋白抑制一般的转录。此外，我们还发现包涵体阳性和阴性神经元的转录水平基本相似。结果表明，包涵体的存在是不是必不可少的抑制一般的转录，相反，它的报道的作用，抑制特定的基因转录的多聚谷氨酰胺疾病的病理。(C)2003年爱思唯尔公司All rights reserved.
By using direct immunocytochemistry of BrU incorporated to RNA in the nuclei, we evaluated the effect of mutant huntingtin and ataxin-1 on general transcription in primary cortical and cerebellar neurons. Our quantitative analyses clearly showed that these mutant polyglutamine disease proteins repress general transcription. In addition, we found that general transcription level was almost similar in inclusion body-positive and -negative neurons. The result suggests that presence of inclusion body is not essential for repressing general transcription in contrast to its reported role for suppressing specific gene transcription in the polyglutamine disease pathology. (C) 2003 Elsevier Inc. All rights reserved.