Kish S J, Perry T L, Hornykiewicz O
J Neurochem. 1984 Feb;42(2):466-9. doi: 10.1111/j.1471-4159.1984.tb02700.x.
Benzodiazepine receptor binding was measured in cerebellar cortex of 15 patients with dominantly inherited olivopontocerebellar atrophy (OPCA). The majority of these patients had a moderate to marked Purkinje cell loss, as judged by the lowered levels of dentate nucleus gamma-aminobutyric acid (GABA), a marker of Purkinje cells. Despite the reduction in Purkinje cell number cerebellar cortical benzodiazepine receptor density was either normal or slightly elevated in the OPCA patients. These results are in contrast to the findings in a mutant strain of mice deficient in Purkinje cells in which the concentration of benzodiazepine receptors in cerebellum is greatly reduced. Our data indicate that in the human, cerebellar cortical benzodiazepine receptors are either not significantly associated with Purkinje cells or that in OPCA Purkinje cell loss triggers a de novo synthesis of extra benzodiazepine binding sites. It is concluded that, in contrast with the rodent, in the human benzodiazepine receptor binding may not serve as a marker for cerebellar Purkinje cells.
在15例常染色体显性遗传性橄榄体脑桥小脑萎缩（OPCA）患者的小脑皮质中检测了苯二氮䓬受体结合情况。根据齿状核γ-氨基丁酸（GABA，一种浦肯野细胞标志物）水平降低判断，这些患者中的大多数存在中度至显著的浦肯野细胞丢失。尽管浦肯野细胞数量减少，但OPCA患者小脑皮质苯二氮䓬受体密度正常或略有升高。这些结果与缺乏浦肯野细胞的小鼠突变品系中的发现形成对比，在该品系中小脑苯二氮䓬受体浓度大幅降低。我们的数据表明，在人类中，小脑皮质苯二氮䓬受体要么与浦肯野细胞无显著关联，要么在OPCA中浦肯野细胞丢失会引发额外苯二氮䓬结合位点的从头合成。得出的结论是，与啮齿动物不同，在人类中苯二氮䓬受体结合可能无法作为小脑浦肯野细胞的标志物。
