Reiner A, Albin R L, Anderson K D, D'Amato C J, Penney J B, Young A B
Department of Anatomy and Neurobiology, University of Tennessee, Memphis 38163.
Proc Natl Acad Sci U S A. 1988 Aug;85(15):5733-7. doi: 10.1073/pnas.85.15.5733.
Huntington disease (HD) is characterized by the loss of striatal 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 findings 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.
亨廷顿舞蹈病（HD）的特征是纹状体投射神经元丧失，而纹状体投射神经元构成了纹状体神经元的绝大多数。为了确定不同群体的纹状体投射神经元之间是否存在差异性丧失，通过免疫组织化学方法，研究了在纹状体靶区域中，源自含P物质和含脑啡肽的不同群体纹状体投射神经元的轴突终末丛的完整性。对17个HD标本的分析表明，在HD的早期和中期，投射到苍白球外侧段的含脑啡肽神经元比投射到苍白球内侧段的含P物质神经元受影响更大。此外，投射到黑质网状部的含P物质神经元比投射到黑质致密部的含P物质神经元受影响更大。在疾病的最晚期，除了投射到黑质致密部的某些纹状体投射明显保留外，所有纹状体靶区域的投射均减少。这些发现可能解释了HD的一些临床表现和药理学特征。它们还可能有助于识别HD潜在的神经缺陷，并提供更多数据来评估当前的HD发病机制模型。
