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The autism-related gene SNRPN regulates cortical and spine development via controlling nuclear receptor Nr4a1
文献类型：期刊论文
| 作者 | Li, HP; Zhao, PP; Xu, Q; Shan, SF; Hu, CC; Qiu, ZL; Xu, X |
| 刊名 | SCIENTIFIC REPORTS
 |
| 出版日期 | 2016 |
| 卷号 | 6页码:29878 |
| 关键词 | POLYPEPTIDE-N SPLICING PROTEIN PRADER-WILLI SMN PROTEIN MOUSE MODEL IN-VIVO EXPRESSION DUPLICATION DENSITY MEMORY |
| 通讯作者 | Xu, X (reprint author), Fudan Univ, Dept Child Hlth Care, Childrens Hosp, 399 Wanyuan Rd, Shanghai 201102, Peoples R China. ; Qiu, ZL (reprint author), Chinese Acad Sci, Shanghai Inst Biol Sci, CAS Ctr Excellence Brain Sci & Intelligence Techn, Inst Neurosci,Key Lab Primate Neurobiol, Shanghai 200031, Peoples R China.,zqiu@ion.ac.cn ; xuxiu@fudan.edu.cn |
| 英文摘要 | The small nuclear ribonucleoprotein polypeptide N (SNRPN) gene, encoding the RNA-associated SmN protein, duplications or deletions of which are strongly associated with neurodevelopmental disabilities. SNRPN-coding protein is highly expressed in the brain. However, the role of SNRPN protein in neural development remains largely unknown. Here we showed that the expression of SNRPN increased markedly during postnatal brain development. Overexpression or knockdown of SNRPN in cortical neurons impaired neurite outgrowth, neuron migration, and the distribution of dendritic spines. We found that SNRPN regulated the expression level of Nr4a1, a critical nuclear receptor during neural development, in cultured primary cortical neurons. The abnormal spine development caused by SNRPN overexpression could be fully rescued by Nr4a1 co-expression. Importantly, we found that either knockdown of Nr4a1 or 3, 3'-Diindolylmethane (DIM), an Nr4a1 antagonist, were able to rescue the effects of SNRPN knockdown on neurite outgrowth of embryonic cortical neurons, providing the potential therapeutic methods for SNRPN deletion disorders. We thus concluded that maintaining the proper level of SNRPN is critical in cortical neurodevelopment. Finally, Nr4a1 may serve as a potential drug target for SNRPN-related neurodevelopmental disabilities, including Prader-Willi syndrome (PWS) and autism spectrum disorders (ASDs). |
| WOS标题词 | Science & Technology - Other Topics |
| 学科主题 | Multidisciplinary Sciences |
| 语种 | 英语 |
| WOS记录号 | WOS:000379878800001 |
| 源URL | [http://ir.sibs.ac.cn/handle/331001/4012]  |
| 专题 | 上海神经科学研究所_神经所(总) |
| 推荐引用方式 GB/T 7714 | Li, HP,Zhao, PP,Xu, Q,et al. The autism-related gene SNRPN regulates cortical and spine development via controlling nuclear receptor Nr4a1[J]. SCIENTIFIC REPORTS,2016,6:29878. |
| APA | Li, HP.,Zhao, PP.,Xu, Q.,Shan, SF.,Hu, CC.,...&Xu, X.(2016).The autism-related gene SNRPN regulates cortical and spine development via controlling nuclear receptor Nr4a1.SCIENTIFIC REPORTS,6,29878. |
| MLA | Li, HP,et al."The autism-related gene SNRPN regulates cortical and spine development via controlling nuclear receptor Nr4a1".SCIENTIFIC REPORTS 6(2016):29878. |
入库方式： OAI收割
来源：上海神经科学研究所
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