Interaction with Polyglutamine-expanded Huntingtin Alters Cellular Distribution and RNA Processing of Huntingtin Yeast Two-hybrid Protein A (HYPA)
文献类型：期刊论文
| 作者 | Jiang, YJ; Che, MX; Yuan, JQ; Xie, YY; Yan, XZ; Hu, HY |
| 刊名 | JOURNAL OF BIOLOGICAL CHEMISTRY
 |
| 出版日期 | 2011 |
| 卷号 | 286期号:28页码:25236-25245 |
| 通讯作者 | Hu, HY (reprint author), Chinese Acad Sci, Shanghai Inst Biol Sci, State Key Lab Mol Biol, Inst Biochem & Cell Biol, Shanghai 200031, Peoples R China.,hyhu@sibs.ac.cn |
| 英文摘要 | Huntington disease (HD) is an autosomal inherited disorder that causes the deterioration of brain cells. The polyglutamine (polyQ) expansion of huntingtin (Htt) is implicated in the pathogenesis of HD via interaction with an RNA splicing factor, Htt yeast two-hybrid protein A/forming-binding protein 11 (HYPA/FBP11). Besides the pathogenic polyQ expansion, Htt also contains a proline-rich region (PRR) located exactly in the C terminus to the polyQ tract. However, how the polyQ expansion influences the PRR-mediated protein interaction and how this abnormal interaction leads to the biological consequence remain elusive. Our NMR structural analysis indicates that the PRR motif of Htt cooperatively interacts with the tandem WW domains of HYPA through domain chaperoning effect of WW1 on WW2. The polyQ-expanded Htt sequesters HYPA to the cytosolic location and then significantly reduces the efficiency of pre-mRNA splicing. We propose that the toxic gain-of-function of the polyQ-expanded Htt that causes dysfunction of cellular RNA processing contributes to the pathogenesis of HD. |
| 学科主题 | Biochemistry & Molecular Biology |
| 类目[WOS] | Biochemistry & Molecular Biology |
| 关键词[WOS] | TANDEM WW DOMAINS ; PROLINE-RICH REGION ; LIGAND RECOGNITION ; GROUP-II/III ; IN-VIVO ; DISEASE ; AGGREGATION ; BINDING ; PATHOGENESIS ; SPECIFICITY |
| 收录类别 | SCI |
| 语种 | 英语 |
| WOS记录号 | WOS:000292547900069 |
| 版本 | 出版稿 |
| 源URL | [http://202.127.25.143/handle/331003/886]  |
| 专题 | 上海生化细胞研究所_上海生科院生化细胞研究所 |
| 推荐引用方式 GB/T 7714 | Jiang, YJ,Che, MX,Yuan, JQ,et al. Interaction with Polyglutamine-expanded Huntingtin Alters Cellular Distribution and RNA Processing of Huntingtin Yeast Two-hybrid Protein A (HYPA)[J]. JOURNAL OF BIOLOGICAL CHEMISTRY,2011,286(28):25236-25245. |
| APA | Jiang, YJ,Che, MX,Yuan, JQ,Xie, YY,Yan, XZ,&Hu, HY.(2011).Interaction with Polyglutamine-expanded Huntingtin Alters Cellular Distribution and RNA Processing of Huntingtin Yeast Two-hybrid Protein A (HYPA).JOURNAL OF BIOLOGICAL CHEMISTRY,286(28),25236-25245. |
| MLA | Jiang, YJ,et al."Interaction with Polyglutamine-expanded Huntingtin Alters Cellular Distribution and RNA Processing of Huntingtin Yeast Two-hybrid Protein A (HYPA)".JOURNAL OF BIOLOGICAL CHEMISTRY 286.28(2011):25236-25245. |
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