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亨廷顿舞蹈症的动物模型。

Animal models of Huntington's disease.

作者信息

Gárdián Gabriella

机构信息

Department of Neurology, University of Szeged.

出版信息

Ideggyogy Sz. 2006 Nov 20;59(11-12):396-9.

Abstract

Huntington's disease is an autosomal dominantly inherited progressive neurodegenerative disorder. The main symptoms are choreiform, involuntary movements, personality changes and dementia. Huntington's disease is a member of a group of diseases caused by CAG repeat expansions. One research aim is to determine the earliest molecular changes associated with Huntington's disease. There is no possibility for this in humans, but various early changes have been identified in an animal model of Huntington's disease. They are constructed by excitotoxin causing striatal lesion, or mitochondrial toxins inducing energy impairment, or by generating transgenic mice.

摘要

亨廷顿舞蹈症是一种常染色体显性遗传的进行性神经退行性疾病。主要症状为舞蹈样、不自主运动、人格改变和痴呆。亨廷顿舞蹈症是由CAG重复序列扩增引起的一组疾病中的一员。一个研究目标是确定与亨廷顿舞蹈症相关的最早分子变化。在人类身上无法做到这一点,但在亨廷顿舞蹈症的动物模型中已发现了各种早期变化。这些模型通过兴奋性毒素导致纹状体损伤、线粒体毒素诱导能量损伤或通过培育转基因小鼠构建而成。

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