Kuśnierz-Alejska G, Duk M, Storry J R, Reid M E, Wiecek B, Seyfried H, Lisowska E
Institute of Haematology and Blood Transfusion, Warsaw, Poland.
Transfusion. 1999 Jan;39(1):32-8. doi: 10.1046/j.1537-2995.1999.39199116892.x.
This report describes the characterization of polyagglutinable red cells (RBCs), identified in two generations of a Polish family.
Untreated and modified RBCs of the proposita (TS) were tested by serologic methods, using human sera, antibodies, lectins, and inhibitors of agglutination. Moreover, glycophorins were characterized by sodium docecyl sulfate-polyacrylamide gel electrophoresis and Western blotting, and glycolipids were purified, fractionated by thin-layer chromatography, and detected with Ricinus communis agglutinin I (RCA-I, specific for galactose residues) and Griffonia simplicifolia IB4 lectin (GSL-IB4, specific for Gal alpha1-3Gal- structure). Some of the experiments were also performed on RBCs of members of TS's family.
Polyagglutination, found in four members of TS's family, was identified as the second case of an earlier described NOR polyagglutination. The polyagglutination was decreased by treating the RBCs with alpha-galactosidase and was inhibited by a neutral glycolipid fraction from NOR+ RBCs. Detection of neutral glycolipids of TS's RBCs on the thin-layer plate by RCA-I and GSL-IB4 revealed the presence of components that were not detectable in control RBCs. Moreover, Western blotting of RBC membranes from five family members with glycophorin monoclonal antibodies and agglutination assays with anti-St(a) and anti-Dantu sera identified the presence of St(a) glycophorin in four members of the family, two of whom were NOR+ and two NOR-.
Our results showed that two rare features of TS's RBCs, NOR polyagglutination and St(a) glycophorin, are inherited independently, and that NOR+ RBCs contain neutral glycolipids with an abnormal oligosaccharide structure, most likely terminated with alpha-galactosyl residues.
本报告描述了在一个波兰家庭的两代人中发现的多凝集红细胞（RBC）的特征。
使用人血清、抗体、凝集素和凝集抑制物，通过血清学方法对先证者（TS）未经处理和经修饰的红细胞进行检测。此外，通过十二烷基硫酸钠-聚丙烯酰胺凝胶电泳和蛋白质印迹对血型糖蛋白进行表征，对糖脂进行纯化，通过薄层色谱进行分离，并用蓖麻凝集素I（RCA-I，对半乳糖残基具有特异性）和西非单叶豆凝集素IB4（GSL-IB4，对Galα1-3Gal结构具有特异性）进行检测。部分实验也在TS家族成员的红细胞上进行。
在TS家族的四名成员中发现的多凝集现象被确定为先前描述的NOR多凝集的第二例。用α-半乳糖苷酶处理红细胞可降低多凝集现象，并且被来自NOR+红细胞的中性糖脂组分所抑制。用RCA-I和GSL-IB4在薄层板上检测TS红细胞的中性糖脂，发现存在对照红细胞中无法检测到的成分。此外，用血型糖蛋白单克隆抗体对五名家族成员的红细胞膜进行蛋白质印迹，并用抗St(a)和抗丹图血清进行凝集试验，确定该家族四名成员中存在St(a)血型糖蛋白，其中两名是NOR+，两名是NOR-。
我们的结果表明，TS红细胞的两个罕见特征，即NOR多凝集和St(a)血型糖蛋白，是独立遗传的，并且NOR+红细胞含有具有异常寡糖结构的中性糖脂，最有可能以α-半乳糖基残基结尾。
