导致遗传性多凝集类型的红细胞抗原。

Red blood cell antigens responsible for inherited types of polyagglutination.

作者信息

Lisowska E, Duk M

机构信息

Department of Immunochemistry, Ludwik Hirszfeld Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, 53-114 Wroclaw, Poland.

出版信息

Adv Exp Med Biol. 2001;491:141-53. doi: 10.1007/978-1-4615-1267-7_11.

DOI:10.1007/978-1-4615-1267-7_11

PMID:14533796

Abstract

The three described types on inheritable polyagglutination are related to altered carbohydrate structures in glycoproteins or/and glycolipds on the erythrocyte surface. HEMPAS, a condition causing anemia and other pathological symptoms, is characterized by impaired biosynthesis of N-glycans, mostly those carried by band 3 and band 4.5 erythrocyte membrane proteins. Cad erythrocytes have abnormal glycophorin O-glycans, structurally related to the more common human Sd(a) and murine CT determinants, and accumulate an Sd(a)-like ganglioside. NOR erythrocytes express recently detected abnormal alpha-galactose-terminated glycosphingolipids, which strongly react with G. simplicifolia IB4 isolectin, but do not react with human anti-Galalpha1-3Gal antibodies.

摘要

所描述的三种遗传性多凝集类型与红细胞表面糖蛋白或/和糖脂中碳水化合物结构的改变有关。遗传性红细胞生成异常性卟啉病（HEMPAS）是一种导致贫血和其他病理症状的疾病，其特征是N-聚糖生物合成受损，主要是由带3和带4.5红细胞膜蛋白携带的那些N-聚糖。Cad红细胞具有异常的血型糖蛋白O-聚糖，其结构与更常见的人类Sd(a)和鼠CT决定簇相关，并积累一种类似Sd(a)的神经节苷脂。NOR红细胞表达最近检测到的异常α-半乳糖末端糖鞘脂，其与简单异叶凝集素IB4强烈反应，但不与人抗-Galα1-3Gal抗体反应。