Selective expression of mutant huntingtin during development recapitulates characteristic features of Huntington's disease

Molero Aldrin E.; Arteaga-Bracho Eduardo E.; Chen Christopher H.Gulinello MariaWinchester Michael L.Pichamoorthy NandiniGokhan SolenKhodakhah KamranMehler Mark F.

首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >Selective expression of mutant huntingtin during development recapitulates characteristic features of Huntington's disease

【24h】

Selective expression of mutant huntingtin during development recapitulates characteristic features of Huntington's disease

机译：Selective expression of mutant huntingtin during development recapitulates characteristic features of Huntington's disease

获取原文

获取原文并翻译 | 示例

掌桥外文数据库（机构版） >>

开具论文收录证明 >>

文献代查 >>

页面导航

摘要
著录项
相关主题

摘要

Recent studies have identified impairments in neural induction and in striatal and cortical neurogenesis in Huntington's disease (HD) knock-in mouse models and associated embryonic stem cell lines. However, the potential role of these developmental alterations for HD pathogenesis and progression is currently unknown. To address this issue, we used BACHD:CAG-Cre(ERT2) mice, which carry mutant huntingtin (mHtt) modified to harbor a floxed exon 1 containing the pathogenic polyglutamine expansion (Q97). Upon tamoxifen administration at postnatal day 21, the floxed mHtt-exon1 was removed and mHtt expression was terminated (Q97(CRE)). These conditional mice displayed similar profiles of impairments to those mice expressing mHtt throughout life: (i) striatal neurodegeneration, (ii) early vulnerability to NMDA-mediated excitotoxicity, (iii) impairments in motor coordination, (iv) temporally distinct abnormalities in striatal electrophysiological activity, and (v) altered corticostriatal functional connectivity and plasticity. These findings strongly suggest that developmental aberrations may play important roles in HD pathogenesis and progression.

著录项

来源
《Proceedings of the National Academy of Sciences of the United States of America》 |2016年第20期|5736-5741|共6页
作者
Molero Aldrin E.; Arteaga-Bracho Eduardo E.; Chen Christopher H.Gulinello MariaWinchester Michael L.Pichamoorthy NandiniGokhan SolenKhodakhah KamranMehler Mark F.;
展开▼
作者单位

Albert Einstein Coll Med, Roslyn & Leslie Goldstein Lab Stem Cell Biol & Re, Bronx, NY 10461 USA;

Albert Einstein Coll Med, Dominick P Purpura Dept Neurosci, Bronx, NY 10461 USA;

Albert Einstein Coll Med, Rose F Kennedy Ctr Res Intellectual & Dev Disabil, Bronx, NY 10461 USAAlbert Einstein Coll Med, Saul R Korey Dept Neurol, Bronx, NY 10461 USA;

展开▼
收录信息美国《科学引文索引》(SCI);美国《生物学医学文摘》(MEDLINE);美国《化学文摘》(CA);
原文格式 PDF
正文语种英语
中图分类自然科学总论;
关键词
neurodegeneration; prodromal; plasticity;

Selective expression of mutant huntingtin during development recapitulates characteristic features of Huntington's disease

摘要

著录项

相关主题

期刊订阅