Fujii Y, Ohno M, Shimada M
Department of Pediatrics, Shiga University of Medical Science, Otsu, Japan.
Brain Dev. 1994 Mar-Apr;16(2):96-103. doi: 10.1016/0387-7604(94)90042-6.
The cerebellum of shaking rat Kawasaki (SRK) was studied histochemically and immunocytochemically. The cerebellar cortex was characterized by a delay in the disappearance of the external granular layer, a narrow molecular layer, a narrow and cell sparse internal granular layer, and disarranged and heterotopically situated Purkinje cells with tortuous arborization and a large cell cluster in the depths of the cerebellum. Golgi-Cox staining revealed an abnormal ramification and polarity of ectopic Purkinje cells. Ultrastructurally, most spines of Purkinje cells in the depths remained naked. Based on these results, it is suggested that the genetically determined mechanisms responsible for the abnormal structure in the cerebellar cortex of SRK may include a migratory disorder of the Purkinje cells, and a decrease in the microneurons with a resulting decrease of synaptic contacts on the Purkinje cell soma and dendrite.
对震颤大鼠川崎病(SRK)的小脑进行了组织化学和免疫细胞化学研究。小脑皮质的特征为外颗粒层消失延迟、分子层狭窄、内颗粒层狭窄且细胞稀疏,以及浦肯野细胞排列紊乱且异位,其树突分支曲折,在小脑深部有大细胞簇。高尔基-考克斯染色显示异位浦肯野细胞有异常分支和极性。超微结构上,深部浦肯野细胞的大多数棘突仍无髓鞘。基于这些结果,提示导致SRK小脑皮质结构异常的遗传决定机制可能包括浦肯野细胞的迁移障碍,以及微神经元减少,从而导致浦肯野细胞体和树突上的突触接触减少。