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亨廷顿舞蹈症模型小鼠的神经生理学评估

Neurophysiological Assessment of Huntington's Disease Model Mice.

作者信息

Donzis Elissa J, Holley Sandra M, Cepeda Carlos, Levine Michael S

机构信息

Intellectual and Developmental Disabilities Research Center, Semel Institute for Neuroscience and Human Behavior, University of California Los Angeles, Los Angeles, CA, USA.

出版信息

Methods Mol Biol. 2018;1780:163-177. doi: 10.1007/978-1-4939-7825-0_9.

DOI:10.1007/978-1-4939-7825-0_9
PMID:29856019
Abstract

Electrophysiological and cell imaging techniques are powerful tools for understanding alterations in neuronal activity in Huntington's disease (HD), a fatal neurological disorder caused by an expansion of CAG repeats in the HTT gene. Changes in neuronal activity often precede the behavioral manifestations of HD, therefore, understanding the electrophysiology of HD is critical for identifying potential prodromal markers and therapeutic targets. This chapter outlines the basic methodology behind four major electrophysiological and imaging techniques used in HD mouse models: patch clamp recordings, optogenetics, in vivo electrophysiology, and Ca imaging, as well as some of the advancements in HD research using each of these techniques.

摘要

电生理和细胞成像技术是理解亨廷顿舞蹈病(HD)神经元活动变化的有力工具,HD是一种由HTT基因中CAG重复序列扩增引起的致命神经疾病。神经元活动的变化通常先于HD的行为表现,因此,了解HD的电生理学对于识别潜在的前驱标志物和治疗靶点至关重要。本章概述了HD小鼠模型中使用的四种主要电生理和成像技术背后的基本方法:膜片钳记录、光遗传学、体内电生理学和钙成像,以及使用这些技术进行HD研究的一些进展。

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Neurophysiological Assessment of Huntington's Disease Model Mice.亨廷顿舞蹈症模型小鼠的神经生理学评估
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Motor Assessment in Huntington's Disease Mice.亨廷顿舞蹈症小鼠的运动评估
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Impaired development of cortico-striatal synaptic connectivity in a cell culture model of Huntington's disease.亨廷顿舞蹈症细胞培养模型中皮质-纹状体突触连接发育受损。
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Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats.亨廷顿舞蹈病140 CAG重复敲入小鼠模型中早期运动和神经病理学异常的时间进程。
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