Identification of glycoprotein storage diseases by lectins: a new diagnostic method.
Identification of glycoprotein storage diseases by lectins: a new diagnostic method.
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凝集素鉴定糖蛋白贮积病：一种新的诊断方法。
DOI：
10.1177/32.12.6501863
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发表时间：
1984
期刊：
影响因子：
--
通讯作者：
M. Pereira
中科院分区：
文献类型：
--
作者：
J. Alroy;U. Orgad;A. Ucci;M. Pereira
糖蛋白储存疾病的具体诊断是通过显示细胞或体液中的酶活性不足或未降解的寡糖升高来作出的。这种研究需要前瞻性采样和昂贵的专业生物化学，这也是耗时的。我们在石蜡包埋的组织切片上使用凝集素试剂来识别未降解储存物质中的特定糖类。我们研究了22例糖蛋白储存疾病，并在组织化学上区分α-和β-甘露糖沉着症、岩藻糖苷沉着症和唾液酸沉着症。感染α-甘露糖症的细胞用刀豆蛋白A(ConA)、普通小麦和琥珀酰甘露糖苷(S-WGA)染色，而β-甘露糖症细胞不用任何凝集素染色。在岩藻糖体病中，受影响的细胞用Ulex Europeus-I(UEA-I)染色，而唾液酸病受影响的细胞用WGA染色，3例用花生(PNA)。本研究表明，凝集素组织化学以一种简单、廉价的方法为某些糖蛋白沉积性疾病提供了一种可靠的特异性诊断模式。
The specific diagnosis of glycoprotein storage diseases is made by demonstrating a deficiency in enzyme activity or an elevation of undegraded oligosaccharides in cells or body fluids. Prospective sampling and expensive specialized biochemistry, which is also time consuming, are required for such studies. We used lectin reagents on paraffin-embedded tissue sections to identify the specific sugars in undegraded stored substances. We studied 22 cases of glycoprotein storage diseases and differentiated histochemically between alpha- and beta-mannosidosis, fucosidosis, and sialisidosis. Cells affected with alpha-mannosidosis stained with Concanavalia ensiformis (Con A), Triticum vulgaris (WGA), and succinyl-WGA (S-WGA), while beta-mannosidosis cells did not stain with any of the lectins used. In fucosidosis the affected cells stained with Ulex europeus-I (UEA-I), while sialisidosis-affected cells stained with WGA, and in three cases with Arachis hypogea (PNA). This study indicates that lectin histochemistry provides a reliable specific diagnostic pattern for some glycoprotein storage diseases using a simple and inexpensive method.