1型脊髓小脑共济失调患者纹状体多巴胺神经末梢标志物减少，但黑质细胞数量未减少。

Striatal dopamine nerve terminal markers but not nigral cellularity are reduced in spinocerebellar ataxia type 1.

作者信息

Kish S J, Guttman M, Robitaille Y, el-Awar M, Chang L J, Levey A I

机构信息

Human Neurochemical Pathology Laboratory, Clarke Institute of Psychiatry, Toronto, ON, Canada.

出版信息

Neurology. 1997 Apr;48(4):1109-11. doi: 10.1212/wnl.48.4.1109.

DOI:10.1212/wnl.48.4.1109

PMID:9109912

Abstract

We previously reported markedly reduced (-76%) dopamine (DA) levels in the putamen of seven patients with spinocerebellar ataxia type 1 (SCA1) who had no evidence of nigral cell loss or parkinsonism. To determine whether the DA reduction was accompanied by loss of DA nerve terminals, we measured levels of the DA transporter ([3H]WIN, 35,428 binding; DA transporter protein) and the vesicular monoamine transporter ([3H]DTBZ binding) in the putamen of these patients. As compared with the controls (n = 14), mean putamen concentrations of [3H]WIN 35,428 binding (-45%), dopamine transporter protein (-61%), and [3H]DTBZ binding (-48%) were significantly reduced in this SCA1 subgroup. We conclude that the degeneration in nigrostriatal DA neurons begins at the nerve ending in SCA1.

摘要

我们之前报道过，在7例1型脊髓小脑共济失调（SCA1）患者的壳核中，多巴胺（DA）水平显著降低（-76%），这些患者没有黑质细胞丢失或帕金森症的证据。为了确定DA水平降低是否伴有DA神经末梢的丢失，我们测量了这些患者壳核中DA转运体（[3H]WIN 35,428结合；DA转运体蛋白）和囊泡单胺转运体（[3H]DTBZ结合）的水平。与对照组（n = 14）相比，该SCA1亚组中[3H]WIN 35,428结合（-45%）、多巴胺转运体蛋白（-61%）和[3H]DTBZ结合（-48%）的壳核平均浓度显著降低。我们得出结论，在SCA1中，黑质纹状体DA神经元的变性始于神经末梢。