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[拉福拉小体、淀粉样体和 Bielschowsky 小体中的所谓细丝。细胞代谢紊乱的胞质内蓄积物质中的束状亚结构(作者译)]

[The so-called filaments in lafora-bodies, corpora amylacea and Bielschowsky-bodies. Fasciolar substructures in accumulated intraplasmatic material of cellular dysmetabolies (author's transl)].

作者信息

Nemetschek T, Volk B, Ule G

出版信息

Acta Neuropathol. 1975 Oct 27;33(1):79-84. doi: 10.1007/BF00685967.

Abstract

Both the corpora amylacea and Lafora bodies have been described in the literature as filamentous structures. On electron microscopic examination they are often composed of ribbon-like fasciolar units. The ultrastructural appearance of these structures is discussed on the example of an amylopectin model. These fasciolar substructures are also encountered in Bielschowsky bodies and are then considered to be a sign of intracellular accumulation of dysmetabolic products. The histological and electron microscopical similarity of corpora amylacea, Lafora bodies and Bielschowsky bodies suggest that all these structures are an unspecific, nearly identical endproduct of various intracellular metabolic disturbances. The pathognomonic significance of these structures depends on their regional distribution: the common corpora amylacea occur predominantly in the astroglia, the Lafora bodies typical in the neurons and the Bielschowsky bodies are restricted to the neurons of the exterior pallidum.

摘要

淀粉样体和拉福拉小体在文献中均被描述为丝状结构。在电子显微镜检查下,它们通常由带状束状单位组成。以支链淀粉模型为例讨论了这些结构的超微结构外观。这些束状亚结构也见于 Bielschowsky 小体,被认为是代谢异常产物细胞内蓄积的标志。淀粉样体、拉福拉小体和 Bielschowsky 小体在组织学和电子显微镜下的相似性表明,所有这些结构都是各种细胞内代谢紊乱的非特异性、几乎相同的终产物。这些结构的病理诊断意义取决于它们的区域分布:常见的淀粉样体主要出现在星形胶质细胞中,典型的拉福拉小体出现在神经元中,而 Bielschowsky 小体仅限于外侧苍白球的神经元。

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