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人类和犬类岩藻糖苷贮积症:一项比较凝集素组织化学研究。

Human and canine fucosidosis: a comparative lectin histochemistry study.

作者信息

Alroy J, Ucci A A, Warren C D

出版信息

Acta Neuropathol. 1985;67(3-4):265-71. doi: 10.1007/BF00687811.

Abstract

Selected formalin-fixed, paraffin-embedded tissues of human and canine fucosidosis were stained with nine different lectins. Neurons, splenic sinusoidal cells, hepatic Kupffer cells, tissue macrophages, and capillary endothelium from human patients with fucosidosis stained intensely with Ulex europaeus agglutinin-I (UEA-I), but the same cells were unstained in tissues from canine fucosidosis. Since UEA-I specifically binds to terminal fucose residues, and fucose-rich undegraded metabolites are stored in affected cells of both human and canine fucosidosis, the variable lectin staining pattern demonstrates an unexpected species-specific histochemical variability. This finding highlights the fact that although both species have decreased fucosidase activity, the precursor substrates, undegraded stored metabolites, and particular cells affected by this enzyme deficiency are different.

摘要

选取人类和犬类岩藻糖苷贮积症的福尔马林固定石蜡包埋组织,用九种不同的凝集素进行染色。人类岩藻糖苷贮积症患者的神经元、脾血窦细胞、肝库普弗细胞、组织巨噬细胞和毛细血管内皮细胞被欧洲荆豆凝集素-I(UEA-I)强烈染色,但犬类岩藻糖苷贮积症组织中的相同细胞未被染色。由于UEA-I特异性结合末端岩藻糖残基,且富含岩藻糖的未降解代谢产物在人类和犬类岩藻糖苷贮积症的受影响细胞中均有储存,这种凝集素染色模式的差异显示出一种意想不到的物种特异性组织化学变异性。这一发现突出了这样一个事实,即尽管两个物种的岩藻糖苷酶活性均降低,但前体底物、未降解的储存代谢产物以及受这种酶缺乏影响的特定细胞是不同的。

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