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肌萎缩侧索硬化症前角细胞中布尼亚小体的超微结构

Ultrastructure of the Bunina bodies in anterior horn cells of amyotrophic lateral sclerosis.

作者信息

Tomonaga M, Saito M, Yoshimura M, Shimada H, Tohgi H

出版信息

Acta Neuropathol. 1978 May 24;42(2):81-6. doi: 10.1007/BF00690971.

Abstract

Light and electron microscopic studies were made on the anterior horn cells in a case o amyotrophic lateral sclerosis. Eosinophilic inclusions of Bunina type were observed almost selectively in the motor neurons of spinal cord, as well as of brain stem, at the light microscopic level. Fine structural study revealed the presence of two types of cytoplasmic inclusions. The first, mainly corresponding to the light microscopic inclusions, were homogeneous, electron-dense, round- or oval-shaped bodies with vesicular or tubular rims and ribosome particles, about 2-5 mu in diameter, which contained filaments or other cytoplasmic componenets in the clear areas within them. The second were lamellar structures (laminated cytoplasmic bodies, Morales) which appeared to be originating from endoplasmic reticulum. There was no distinct transition in these two types of inclusions and the relationship to each other is not clear. The significance of Bunina body is unknown, but some manifestation of a primary disorder, e.g., protein metabolism, rather than a secondary degenerative change in the motor neurons in amyotorophic lateral sclerosis.

摘要

对一例肌萎缩侧索硬化症患者的前角细胞进行了光镜和电镜研究。在光镜水平上,几乎仅在脊髓以及脑干的运动神经元中观察到了布尼纳型嗜酸性包涵体。精细结构研究揭示存在两种类型的胞质包涵体。第一种主要对应于光镜下的包涵体,是均匀的、电子致密的、圆形或椭圆形的物体,有泡状或管状边缘以及核糖体颗粒,直径约2 - 5微米,其在内部的透明区域含有细丝或其他胞质成分。第二种是层状结构(层状胞质体,莫拉莱斯氏体),似乎起源于内质网。这两种类型的包涵体之间没有明显的过渡,它们彼此之间的关系尚不清楚。布尼纳小体的意义尚不清楚,但它可能是原发性疾病(例如蛋白质代谢)的某种表现,而非肌萎缩侧索硬化症中运动神经元的继发性退行性改变。

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