An ultrastructural and experimental study on the development of tubular structures in the lysosomes of Gaucher cells.
An ultrastructural and experimental study on the development of tubular structures in the lysosomes of Gaucher cells.
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关于戈谢细胞溶酶体管状结构发育的超微结构和实验研究。
DOI：
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发表时间：
1988
期刊：
影响因子：
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通讯作者：
H. Hojo
中科院分区：
文献类型：
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作者：
M. Naito;Kiyoshi Takahashi;H. Hojo
戈谢氏细胞特有的储存包涵体含有许多管状结构，并被证明是酶细胞化学转化的次级溶酶体。管状结构主要由扭曲的多层和部分有或无扭曲的平面层组成。从戈谢氏脾脏中提取和纯化的糖脑苷类似于这些平面层。因此，平面层被认为是糖脑苷分子结晶形成的管状结构的基本精细结构单元。电镜观察到，在血细胞，特别是红细胞的消化过程中，在戈歇细胞的溶酶体中形成管状结构。在一项培养实验中，从戈谢病患者获得的单核细胞在摄入热变性红细胞后，在吞噬溶酶体中形成管状结构，成为戈谢病细胞。在另一个培养实验中，单核细胞也通过在培养基中加入管状结构转化为戈谢细胞。因此，巨噬细胞溶酶体内形成管状结构以及巨噬细胞摄取其他戈谢细胞释放的细胞外管状结构似乎对戈谢细胞的发育至关重要。
Characteristic storage inclusions of Gaucher cells contain numerous tubular structures and are demonstrated enzyme-cytochemically to be transformed secondary lysosomes. Tubular structures are composed mainly of twisted multilayers and partially of flat layers with or without twisting. Glucocerebroside extracted and purified biochemically from the Gaucher spleen resembles these flat layers. Therefore, the flat layers are considered to be a fundamental fine structural unit of the tubular structures made by crystallization of glucocerebroside molecules. The tubular structures are observed by electron microscopy to be formed in the digestive process of blood cells, especially erythrocytes, in the lysosomes of Gaucher cells. In one culture experiment, monocytes obtained from Gaucher patients developed tubular structures in the phagolysosomes after ingesting heat-denatured erythrocytes and became Gaucher cells. In the other culture experiment, monocytes were also transformed into Gaucher cells by taking up tubular structures added to the culture medium. Thus, both the intralysosomal formation of tubular structures in the macrophages and their ingestion of extracellular tubular structures released from other Gaucher cells seem essential for the development of Gaucher cells.