Genetically engineered mouse models of neurodegenerative diseases

被引：173

作者：

Wong, PC

Cai, HB

Borchelt, DR

Price, DL

机构：

[1] Johns Hopkins Univ, Sch Med, Dept Pathol, Baltimore, MD 21205 USA

[2] Johns Hopkins Univ, Sch Med, Dept Neurosci, Baltimore, MD 21205 USA

[3] Johns Hopkins Univ, Sch Med, Dept Neurol, Baltimore, MD 21205 USA

[4] Johns Hopkins Univ, Sch Med, Div Neuropathol, Baltimore, MD 21205 USA

来源：

NATURE NEUROSCIENCE | 2002年 / 5卷 / 07期

关键词：

D O I：

10.1038/nn0702-633

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

Recent research has significantly advanced our understanding of the molecular mechanisms of neurodegenerative diseases, including Alzheimer's disease (AD) and motor neuron disease. Here we emphasize the use of genetically engineered mouse models that are instrumental for understanding why AD is a neuronal disease, and for validating attractive therapeutic targets. In motor neuron diseases, Cu/Zn superoxide dismutase and survival motor neuron mouse models are useful in testing disease mechanisms and therapeutic strategies for amyotrophic lateral sclerosis (ALS) and spinal motor atrophy, respectively, but the mechanisms that account for selective motor neuron loss remain uncertain. We anticipate that, in the future, therapies based on understanding disease mechanisms will be identified and tested in mouse model systems.

引用

页码：633 / 639

页数：7

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