Interaction between Mutant Ataxin-1 and PQBP-1 Affects Transcription and Cell Death.

Okazawa H; Rich T; Chang A; Lin X; Waragai M; Kajikawa M; Enokido Y; Komuro A; Kato S; Shibata M; Hatanaka H; Mouradian MM; Sudol M; Kanazawa I

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Interaction between Mutant Ataxin-1 and PQBP-1 Affects Transcription and Cell Death.

机译：突变Ataxin-1和PQBP-1之间的相互作用影响转录和细胞死亡。

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PQBP-1 was isolated on the basis of its interaction with polyglutamine tracts. In this study, using in vitro and in vivo assays, we show that the association between ataxin-1 and PQBP-1 is positively influenced by expanded polyglutamine sequences. In cell lines, interaction between the two molecules induces apoptotic cell death. As a possible mechanism underlying this phenomenon, we found that mutant ataxin-1 enhances binding of PQBP-1 to the C-terminal domain of RNA polymerase II large subunit (Pol II). This reduces the level of phosphorylated Pol II and transcription. Our results suggest the involvement of PQBP-1 in the pathology of spinocerebellar ataxia type 1 (SCA1) and support the idea that modified transcription underlies polyglutamine-mediated pathology.

机译：PQBP-1是基于其与聚谷氨酰胺束的相互作用而分离的。在这项研究中，使用体外和体内试验，我们显示了紫杉醇-1和PQBP-1之间的关联受到扩展的聚谷氨酰胺序列的积极影响。在细胞系中，两个分子之间的相互作用诱导凋亡性细胞死亡。作为这种现象的潜在机制，我们发现突变型共青素-1增强了PQBP-1与RNA聚合酶II大亚基（Pol II）C端结构域的结合。这降低了磷酸化的Pol II和转录水平。我们的结果表明PQBP-1参与1型脊髓小脑共济失调的病理学（SCA1），并支持修饰的转录是多谷氨酰胺介导的病理学基础的观点。

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来源
《Neuron》 |2002年第5期|共13页
作者
Okazawa H; Rich T; Chang A; Lin X; Waragai M; Kajikawa M; Enokido Y; Komuro A; Kato S; Shibata M; Hatanaka H; Mouradian MM; Sudol M; Kanazawa I;
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正文语种 eng
中图分类神经生理学;
关键词
polyglutamine; Cell Death; Drug Interactions; Pathology processes; ataxin-1; 细胞死亡; 药物相互作用;

机译：polyglutamine;Cell Death;Drug Interactions;Pathology processes;ataxin-1;细胞死亡;药物相互作用;

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专利

1. Interaction between Mutant Ataxin-1 and PQBP-1 Affects Transcription and Cell Death. [J] . Okazawa H, Rich T, Chang A, Neuron . 2002,第5期

机译：突变Ataxin-1和PQBP-1之间的相互作用影响转录和细胞死亡。
2. SUMO-1 interacts with mutant ataxin-1 and colocalizes to its aggregates in purkinje cells of SCA1 transgenic mice [J] . Kang S., Hong S. Archives Italiennes de Biologie . 2010,第4期

机译：SUMO-1与突变型紫杉素-1相互作用并共定位于SCA1转基因小鼠的浦肯野细胞中的聚集体
3. Differential Transcriptional Activation by Human T-Cell Leukemia Virus Type 1 Tax Mutants Is Mediated by Distinct Interactions with CREB Binding Protein and p300 [J] . Fran?oise Bex, Min-Jean Yin, Arsène Burny, Molecular and Cellular Biology . 1998,第4期

机译：通过与CREB结合蛋白和p300的独特相互作用介导的人类T细胞白血病病毒1型税收突变体的差异转录激活。
4. FLIM-FRET Microscopy to Visualize Transcription Factor Interactions in the Nucleus of the Living Cell [C] . Richard N. Day, Ignacio A. Demarco, Ty C. Voss, Conference on Multiphoton Microscopy in the Biomedical Sciences IV; 20040125-20040127; San Jose,CA; US . 2004

机译：FLIM-FRET显微镜观察活细胞核中转录因子的相互作用
5. Interactions of shiga-like toxin-2 (Stx-2) from Escherichia coli O157:H7 and the Bcl-2 family of proteins during host cell programmed cell death. [D] . Jeffrey, Lia K. 2013

机译：大肠杆菌O157：H7的志贺样毒素2（Stx-2）与Bcl-2家族蛋白在宿主细胞程序性细胞死亡期间的相互作用。
6. Suppression of the novel ER protein Maxer by mutant ataxin-1 in Bergman glia contributes to non-cell-autonomous toxicity [O] . Hiroki Shiwaku, Natsue Yoshimura, Takuya Tamura, 2010

机译：Bergman胶质细胞中突变的共济失调素-1抑制新型ER蛋白Maxer有助于非细胞自主毒性
7. Interaction between Mutant Ataxin-1 and PQBP-1 Affects Transcription and Cell Death [O] . Okazawa Hitoshi, Rich Tina, Chang Alex, 2002

机译：突变Ataxin-1和PQBP-1之间的相互作用影响转录和细胞死亡。

Interaction between Mutant Ataxin-1 and PQBP-1 Affects Transcription and Cell Death.

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