Genetic neurodegenerative diseases: The human illness and transgenic models

被引：216

作者：

Price, DL

Sisodia, SS

Borchelt, DR

机构：

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

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

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

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

[5] Univ Chicago, Dept Pharmacol & Physiol Sci, Chicago, IL 60637 USA

来源：

SCIENCE | 1998年 / 282卷 / 5391期

关键词：

D O I：

10.1126/science.282.5391.1079

中图分类号：

O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

The neurodegenerative disorders, a heterogeneous group of chronic progressive diseases, are among the most puzzling and devastating illnesses in medicine. Some of these disorders, such as Alzheimer's disease, amyotrophic lateral sclerosis, the prion diseases, and Parkinson's disease, can occur sporadically and, in some instances, are caused by inheritance of gene mutations. Huntington's disease is acquired in an entirely genetic manner. Transgenic mice that express disease-causing genes recapitulate many features of these diseases. This review provides an overview of transgenic mouse models of familiar amyotrophic lateral sclerosis, familiar Alzheimer's disease, and Huntington's disease and the emerging insights relevant to the underlying molecular mechanisms of these diseases.

引用

页码：1079 / 1083

页数：5

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