Kish S J, Shannak K S, Perry T L, Hornykiewicz O
J Neurochem. 1983 Nov;41(5):1495-7. doi: 10.1111/j.1471-4159.1983.tb00852.x.
Alterations in one subunit of the proposed GABA receptor complex, namely, the GABA receptor, have been observed in Huntington's disease cerebellum. We measured binding to a second subunit, the benzodiazepine binding site, in the autopsied cerebellum of 12 patients dying with adult-onset Huntington's disease. Neuronal benzodiazepine ([3H]flunitrazepam) binding density (Bmax) and affinity in cerebellar cortex of the Huntington's disease patients were not significantly different from control values. Similarly, maximal GABA stimulation of benzodiazepine binding was normal in the Huntington's disease cerebellum. In addition, no significant changes were observed in the concentrations of GABA, glutamate, and taurine in cerebellar cortex, nor of GABA in the dentate nucleus.
在亨廷顿舞蹈病患者的小脑中，已观察到假定的GABA受体复合物的一个亚基（即GABA受体）发生了改变。我们检测了12例成年期发病的亨廷顿舞蹈病患者尸检小脑标本中与第二个亚基（苯二氮䓬结合位点）的结合情况。亨廷顿舞蹈病患者小脑皮质中的神经元苯二氮䓬（[3H]氟硝西泮）结合密度（Bmax）和亲和力与对照值无显著差异。同样，在亨廷顿舞蹈病患者的小脑中，GABA对苯二氮䓬结合的最大刺激作用也正常。此外，小脑皮质中GABA、谷氨酸和牛磺酸的浓度，以及齿状核中GABA的浓度均未观察到显著变化。
