Involvement of lysosomes in the pathogenesis of CAG repeat diseases

被引：18
|
作者：
Yamada, M
Tsuji, S
Takahashi, H
机构：
[1] Niigata Univ, Brain Res Inst, Dept Pathol, Niigata 9518585, Japan
[2] Niigata Univ, Brain Res Inst, Dept Neurol, Niigata 9518585, Japan
来源：
ANNALS OF NEUROLOGY | 2002年 / 52卷 / 04期
关键词：
D O I：
10.1002/ana.10328
中图分类号：
R74 [神经病学与精神病学];
学科分类号：
摘要：
In CAG repeat diseases, affected neurons possess many cytoplasmic granules immunopositive for expanded polyglutamine stretches. Electron microscopic immunohistochemistry showed that the granules corresponded to lysosomes of primitive type. The results suggest that, in addition to the ubiquitin/proteasome pathway, mutant proteins with expanded polyglutamine stretches are involved in the lysosomal pathway for protein degradation and that this processing mechanism may serve as a target for a new therapeutic approach to CAG repeat diseases.
引用
收藏
页码：498 / 503
页数：6
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