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黏多糖贮积症中脂褐素在神经元内的储存。关于1例具有组织化学和电子显微镜检查结果的Sanfilippo综合征病例的报告。

Storage of lipofuscin in neurons in mucopolysaccharidosis. Report on a case of Sanfilippo's syndrome with histochemical and electron-microscopic findings.

作者信息

Oldfors A, Sourander P

出版信息

Acta Neuropathol. 1981;54(4):287-92. doi: 10.1007/BF00697002.

DOI:10.1007/BF00697002
PMID:6267869
Abstract

A histochemical and ultrastructural study was made on the brain of a 23-year-old man with Sanfilippo's syndrome. In accordance with previous reports the cortical nerve cells contained a PAS-positive lipid storage substance. This showed intense autofluorescence in UV-light and was positive with various stains for lipofuscin. The storage material appeared ultrastructurally as inclusion bodies composed of short lamellated membranes, granular material, and vacuoles. In addition, concentrically and transversely lamellated membranous cytoplasmic bodies were observed in the nerve cells. It is concluded that the PAS-positive lipid storage material in the neurons was composed partly of lipofuscin in addition to other lipids presumably glycosphingolipids.

摘要

对一名患有桑菲利波综合征的23岁男性的大脑进行了组织化学和超微结构研究。与先前的报告一致，皮质神经细胞含有一种PAS阳性脂质储存物质。该物质在紫外线下显示出强烈的自发荧光，并且对脂褐素的各种染色呈阳性。储存物质在超微结构上表现为由短的层状膜、颗粒物质和液泡组成的包涵体。此外，在神经细胞中观察到同心和横向层状的膜性细胞质体。结论是，神经元中PAS阳性脂质储存物质除了其他可能是糖鞘脂的脂质外，部分由脂褐素组成。

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4
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Mucopolysaccharidosis type 3 (Sanfilippo's syndrome).3型黏多糖贮积症（桑菲利波综合征）。
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[Mucopolysaccharidosis type 3 (Sanfilippo's syndrome). A cause of progressive mental retardation].[黏多糖贮积症Ⅲ型（Sanfilippo综合征）。进行性智力发育迟缓的一个病因]
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