Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice
Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice
复制标题
亨廷顿病转基因小鼠中神经纤维聚集体的超微结构定位和渐进形成
DOI：
10.1093/hmg/8.7.1227
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发表时间：
1999-07-01
影响因子：
3.5
通讯作者：
Li, XJ
中科院分区：
文献类型：
--
作者：
Li, H;Li, SH;Li, XJ
How aggregates of polyglutamine proteins, are involved in the neurological symptoms of glutamine repeat diseases is unknown. We show that huntingtin aggregates are present in the neuronal processes of transgenic mice that express exon 1 of the Huntington's disease (HD) gene. Unlike aggregates in the nucleus, these neuropil aggregates are usually smaller and are not ubiquitinated. Electron microscopy reveals many neuropil aggregates in axons and axon terminals. Huntingtin aggregates in the axon terminal are colocalized with some synaptic vesicles, implying that they may affect synaptic transmission and neuronal communication. The formation of neuropil aggregates is highly correlated with the development of neurological symptoms. The present study raises the possibility that neuropil aggregates may cause a dysfunction in neuronal communication and contribute to the neurological symptoms of HD.
