Kanazawa I
Department of Neurology, University of Tokyo.
Rinsho Shinkeigaku. 1993 Dec;33(12):1288-90.
Spinocerebellar degeneration (SCD) is one of the most frequent neurodegenerative disorders in Japan. In spite of this, only a little attention has been paid on the disease, especially from the biochemical point of view. In this symposium, I would like to briefly review the fruits of biochemical researches in Japan. GABA, glutamate and aspartate in the cerebellar system of SCD patients disclosed decreases corresponding to the neuronal cell loss in, for instance, the dentate nucleus for GABA. However, it is interesting to know that in case of choline acetylase, its activity in the cerebellar system in CCA increases greatly corresponding to the reservation of cholinergic ponto-cerebellar system. Since the ponto-cerebellar system in CCA is morphologically preserved, this may be a reflection of a compensatory response to the degeneration of cortical cerebellar systems. Finally, I would like to demonstrate a significant decrease of homocysteic acid concentrations in the cerebellar cortical tissues, but not in olive, dentate in OPCA.
脊髓小脑变性（SCD）是日本最常见的神经退行性疾病之一。尽管如此，人们对这种疾病的关注却很少，尤其是从生物化学角度。在本次研讨会上，我想简要回顾一下日本生物化学研究的成果。SCD患者小脑系统中的γ-氨基丁酸（GABA）、谷氨酸和天冬氨酸含量下降，这与例如齿状核中GABA神经元细胞的损失相对应。然而，有趣的是，在胆碱乙酰化酶的情况下，其在CCA小脑系统中的活性与胆碱能脑桥小脑系统的保留相对应而大幅增加。由于CCA中的脑桥小脑系统在形态上得以保留，这可能是对皮质小脑系统退化的一种代偿反应。最后，我想展示一下在OPCA患者中，小脑皮质组织中的高半胱氨酸浓度显著下降，但橄榄核、齿状核中则不然。
