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强直性肌营养不良中丘脑神经元包涵体的超微结构

Ultrastructure of thalamic neuronal inclusions in myotonic dystrophy.

作者信息

Wiśniewski H M, Berry K, Spiro A J

出版信息

J Neurol Sci. 1975 Mar;24(3):321-9. doi: 10.1016/0022-510x(75)90252-x.

Abstract

Recently, the presence of thalamic neuronal cytoplasmic inclusions in patients with myotonic dystrophy has been reported. At the ultrastructural level, the inclusions were described "containing a fibrillar material within a limiting membrane studded on its outer surface with ribosomes". We have studied the brain of a 48-year-old woman with myotonic dystrophy. Many neuronal inclusion bodies were found within the thalamus, and examined in the electron microscope. Inclusions were found to have an electron density resembling that of nuclear chromatin, were not membrane-bound, and had an internal structure composed of parallel alternating dark and pale lines. We confirm the previously-reported existence of cytoplasmic thalamic neuronal inclusions in myotonic dystrophy, but differ in our observations of their ultrastructural appearance, and note that these inclusions bear no resemblance to previously described inclusion bodies.

摘要

最近,有报道称在强直性肌营养不良患者中存在丘脑神经元胞质内包涵体。在超微结构水平上,这些包涵体被描述为“在其外表面镶嵌有核糖体的界限膜内含有纤维状物质”。我们研究了一名48岁强直性肌营养不良女性的大脑。在丘脑中发现了许多神经元包涵体,并进行了电子显微镜检查。发现包涵体具有类似于核染色质的电子密度,无膜包被,其内部结构由平行交替的明暗线条组成。我们证实了先前报道的强直性肌营养不良中丘脑神经元胞质内包涵体的存在,但在其超微结构外观的观察上存在差异,并指出这些包涵体与先前描述的包涵体没有相似之处。

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