个人科研网厅 | | 帮助
首都医科大学宣武医院知识库
高级检索
当前位置： 首页 > 详情页

A novel and accurate full-length HTT mouse model for Huntington's disease.

| 认领 | 导出 | |

文献详情

资源类型：
WOS体系：
Pubmed体系：

收录情况： ◇ SCIE ◇ 自然指数

作者：
Shenoy Sushila A[1] * ; Zheng Sushuang[2] * ; Liu Wencheng[1] ; Dai Yuanyi[2] ; Liu Yuanxiu[2] ; Hou Zhipeng[3] ; Mori Susumu[3] ; Tang Yi[4] ; Cheng Jerry[5] ; Duan Wenzhen[6] ; Li Chenjian[2] ;
机构： [1]Department of Neuroscience, Weill Cornell Graduate School of Medical Sciences, New York, United States. [2]The MOE Key Laboratory of Cell Proliferation and Differentiation, School of Life Sciences, Peking University, Beijing, China. [3]The Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, Baltimore, United States. [4]Innovation Center for Neurological Disorders, Department of Neurology, Xuanwu Hospital Capital Medical University, National Center for Neurological Disorders, Beijing, China. [5]Department of Computer Science, New York Institute of Technology, New York, United States. [6]Division of Neurobiology, Department of Psychiatry and Behavioral Sciences ,Solomon H.Snyder Department of Neuroscience, Johns Hopkins University School of medicine, Baltimore, United States.
出处：
DOI： 10.7554/eLife.70217
ISSN：

摘要：
Here, we report the generation and characterization of a novel Huntington's disease (HD) mouse model BAC226Q by using a bacterial artificial chromosome (BAC) system, expressing full-length human HTT with ~226 CAG-CAA repeats and containing endogenous human HTT promoter and regulatory elements. BAC226Q recapitulated a full-spectrum of age-dependent and progressive HD-like phenotypes without unwanted and erroneous phenotypes. BAC226Q mice developed normally, and gradually exhibited HD-like psychiatric and cognitive phenotypes at 2 months. From 3 to 4 months, BAC226Q mice showed robust progressive motor deficits. At 11 months, BAC226Q mice showed significant reduced life span, gradual weight loss and exhibited neuropathology including significant brain atrophy specific to striatum and cortex, striatal neuronal death, widespread huntingtin inclusions, and reactive pathology. Therefore, the novel BAC226Q mouse accurately recapitulating robust, age-dependent, progressive HD-like phenotypes will be a valuable tool for studying disease mechanisms, identifying biomarkers, and testing gene-targeting therapeutic approaches for HD.© 2022, Shenoy et al.

基金：
This project was partially supported by Hereditary Disease Foundation, Weill Cornell Medical College, and Peking University School of Life Sciences. The authors wish to thank the National Center for Protein Sciences at Peking University for technical assistance. The authors declare no competing financial interests.
语种：
外文
被引次数：
WOS：
PubmedID：
中科院(CAS)分区：
出版当年[2021]版：
大类 | 1 区 生物学
小类 | 1 区 生物学
最新[2023]版：
大类 | 1 区 生物学
小类 | 1 区 生物学
JCR分区：
出版当年[2020]版：
Q1 BIOLOGY
最新[2023]版：
Q1 BIOLOGY

影响因子： 最新[2023版] 最新五年平均 出版当年[2020版] 出版当年五年平均 出版前一年[2019版] 出版后一年[2021版]

第一作者：
第一作者机构： [1]Department of Neuroscience, Weill Cornell Graduate School of Medical Sciences, New York, United States.
共同第一作者：
通讯作者：
推荐引用方式(GB/T 7714)：
Shenoy Sushila A,Zheng Sushuang,Liu Wencheng,et al.A novel and accurate full-length HTT mouse model for Huntington's disease.[J].ELIFE.2022,11:doi:10.7554/eLife.70217.
APA：
Shenoy Sushila A,Zheng Sushuang,Liu Wencheng,Dai Yuanyi,Liu Yuanxiu...&Li Chenjian.(2022).A novel and accurate full-length HTT mouse model for Huntington's disease..ELIFE,11,
MLA：
Shenoy Sushila A,et al."A novel and accurate full-length HTT mouse model for Huntington's disease.".ELIFE 11.(2022)

资源点击量：16461 今日访问量：0 总访问量：871 更新日期：2025-01-01 建议使用谷歌、火狐浏览器 常见问题

版权所有©2020 首都医科大学宣武医院 技术支持：重庆聚合科技有限公司 地址：北京市西城区长椿街45号宣武医院