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亨廷顿舞蹈症的小鼠模型

Mouse Models of Huntington's Disease.

作者信息

Farshim Pamela P, Bates Gillian P

机构信息

Department of Neurodegenerative Disease, Huntington's Disease Centre and Dementia Research Institute, University College London Institute of Neurology, London, WC1N 3BG, UK.

出版信息

Methods Mol Biol. 2018;1780:97-120. doi: 10.1007/978-1-4939-7825-0_6.

Abstract

The identification of the mutation causing Huntington's disease (HD) has led to the generation of a large number of mouse models. These models are used to further enhance our understanding of the mechanisms underlying the disease, as well as investigating and identifying therapeutic targets for this disorder. Here we review the transgenic, knock-in mice commonly used to model HD, as well those that have been generated to study specific disease mechanisms. We then provide a brief overview of the importance of standardizing the use of HD mice and describe brief protocols used for genotyping the mouse models used within the Bates Laboratory.

摘要

亨廷顿舞蹈症(HD)致病突变的鉴定催生了大量小鼠模型。这些模型用于进一步加深我们对该疾病潜在机制的理解,以及研究和确定针对这种病症的治疗靶点。在此,我们综述了常用于模拟HD的转基因、基因敲入小鼠,以及为研究特定疾病机制而构建的小鼠模型。然后,我们简要概述了规范使用HD小鼠的重要性,并介绍了贝茨实验室用于对所使用的小鼠模型进行基因分型的简要方案。

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