Mechanism of neurodegenerative disease: role of the ubiquitin proteasome system

被引：98

作者：

Petrucelli, L

Dawson, TM

机构：

[1] Johns Hopkins Univ, Sch Med, Inst Cell Engn, Dept Neurol, Baltimore, MD 21205 USA

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

[3] Mayo Clin Jacksonville, Jacksonville, FL USA

来源：

ANNALS OF MEDICINE | 2004年 / 36卷 / 04期

关键词：

alpha-synuclein; neurodegeneration; Parkinson's disease; parkin; proteasome; ubiquitin;

D O I：

10.1080/07853890410031948

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Many neurodegenerative disorders such as Alzheimer's disease (AD) Parkinson's disease (PD), amyotrophic lateral sclerosis (ALS) and Huntington's disease (HD) are characterized by neuronal damage that may be caused by toxic, abnormal, aggregation-prone proteins. The purpose of this review is threefold: 1) to provide the reader with an overview of the genes involved in the abnormal processing and accumulation of misfolded proteins in neurodegenerative diseases using PD as a model disease; 2) to understand the cellular mechanisms for disposal of abnormal proteins, and the effects of toxic protein accumulation on ubiquitin proteasome system (UPS) and neuronal survival and 3) to discuss the development and challenges of cell culture and animal models for a rational and effective treatment for these disorders.

引用

收藏

页码：315 / 320

页数：6

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