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大脑蛋白病:蛋白质构象和组装的神经退行性疾病。

The cerebral proteopathies: neurodegenerative disorders of protein conformation and assembly.

作者信息

Walker L C, LeVine H

机构信息

Neuroscience Therapeutics, Pfizer Ann Arbor Laboratories, MI 48105, USA.

出版信息

Mol Neurobiol. 2000 Feb-Apr;21(1-2):83-95. doi: 10.1385/MN:21:1-2:083.

Abstract

The abnormal assembly and deposition of specific proteins in the brain is the probable cause of most neurodegenerative disease afflicting the elderly. These "cerebral proteopathies" include Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), prion diseases, and a variety of other disorders. Evidence is accumulating that the anomalous aggregation of the proteins, and not a loss of protein function, is central to the pathogenesis of these diseases. Thus, therapeutic strategies that reduce the production, accumulation, or polymerization of pathogenic proteins might be applicable to a wide range of some of the most devastating diseases of old age.

摘要

大脑中特定蛋白质的异常组装和沉积可能是困扰老年人的大多数神经退行性疾病的病因。这些“脑蛋白病”包括阿尔茨海默病(AD)、帕金森病(PD)、亨廷顿病(HD)、朊病毒病以及其他多种疾病。越来越多的证据表明,蛋白质的异常聚集而非蛋白质功能丧失是这些疾病发病机制的核心。因此,减少致病蛋白质产生、积累或聚合的治疗策略可能适用于一些老年期最具破坏性的广泛疾病。

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