DIFFERENTIAL LOSS OF STRIATAL PROJECTION NEURONS IN HUNTINGTON DISEASE
DIFFERENTIAL LOSS OF STRIATAL PROJECTION NEURONS IN HUNTINGTON DISEASE
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DOI：
10.1073/pnas.85.15.5733
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发表时间：
1988-08-01
影响因子：
11.1
通讯作者：
YOUNG, AB
中科院分区：
文献类型：
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作者：
REINER, A;ALBIN, RL;YOUNG, AB
亨廷顿病（HD）的特征在于纹状体投射神经元的丧失，所述纹状体投射神经元构成纹状体神经元的绝大多数。为了确定是否有不同的纹状体投射神经元的群体之间的差异损失，轴突终末丛的完整性所产生的不同群体的P物质含有和脑啡肽纹状体投射神经元进行了研究，在纹状体的目标区域通过免疫组织化学。对17例HD标本的分析表明，在HD的早期和中期，投射到苍白球外段的含脑啡肽的神经元比投射到苍白球内段的含P物质的神经元受到更大的影响。此外，含有P物质的神经元投射到黑质网状部比那些投射到黑质网状部的影响更大。在疾病的最晚期阶段，所有纹状体靶区的投射都被耗尽，除了一些明显保留的纹状体投射到黑质部的外。这些发现可以解释HD的一些临床表现和药理学。它们还可以帮助识别HD潜在的神经缺陷，并提供额外的数据来评估HD发病机制的当前模型。
Huntington disease (HD) is characterized by the loss of striatial projection neurons, which constitute the vast majority of striatal neurons. To determine whether there is differential loss among different populations of striatal projection neurons, the integrity of the axon terminal plexuses arising from the different populations of substance P-containing and enkephalin-containing striatal projection neurons was studied in striatal target areas by immunohistochemistry. Analysis of 17 HD specimens indicated that in early and middle stages of HD, enkephalin-containing neurons projecting to the external segment of the globus pallidus were much more affected than substance P-containing neurons projecting to the internal pallidal segment. Furthermore, substance P-containing neurons projecting to the substantia nigra pars reticulata were more affected than those projecting to the substantia nigra pars compacta. At the most advanced stages of the disease, projections to all striatal target areas were depleted, with the exception of some apparent sparing of the striatal projection to the substantia nigra pars compacta. These finding may explain some of the clinical manifestations and pharmacology of HD. They also may aid in identifying the neural defect underlying HD and provide additional data with which to evaluate current models of HD pathogenesis.