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红细胞膜上的Ge血型系统抗原位于唾液酸糖蛋白的膜糖蛋白C(GP C)和GP D上,它们是同一基因(GYPC)的产物。两种稀有的红细胞Ge表现型Ge:-2,3,4和Ge:-2,-3,4携带的GPC变异体分别命名为GPC.Yus和GPC.Ge。用糖元染色PAS或免疫印迹法能够检测这两个变异体,在GPC和GPD之间存在宽弥散带Mr。GPC.Yus对胰蛋白酶敏感而GPC.Ge却不敏感。病例研究:爱尔兰成年男病人DJL,因结肠肿瘤手术配血,其血清和所有献血者红细胞不相容。DJL的红细胞表型为Ge:-2,-3,-4,血清中含抗-Ge2(+P1)。找不到Ge-的献血者。有趣的是DJL两个孩子的红细胞和DJL的血清相配合。然后进行了家系调查。结果:血清学用未处理与胰蛋白酶处理的细胞及红细胞膜免疫印迹法证实以下的Ge表现型和基因型:DJL(Ge-):Ge:-2,-3,4 GYPC.Ge/GYPC.Ge;配偶(Ge+):Ge:2,3,4 GYPC/GYPC.Yus;2个孩子为(Ge+):Ge:2,3,4 GYPC/GYPC.Ge;6个孩子为(Ge-):Ge:-2,3,4 GYPC.Ge/GYPC.Yus。作者认为:这个研究表明在一个家庭中有两种
The Ge blood group system antigens on the erythrocyte membrane are located on the membrane glycoprotein C (GP C) and GP D of sialic acid glycoproteins, which are the products of the same gene (GYPC). Two rare erythrocyte Ge phenotypes Ge: -2,3,4 and Ge: -2, -3,4 carry GPC variants named GPC.Yus and GPC.Ge. respectively. Both variants were detected by glycogen-stained PAS or immunoblot with wide dispersion band between GPC and GPD GPC.Yus is trypsin sensitive and GPC.Ge is not sensitive. Case Study: Irish adult male patient DJL, due to colon tumor surgery with blood, the serum and all blood donors red blood cells are incompatible. The erythrocyte phenotype of DJL is Ge: -2, -3, -4, and serum contains anti-Ge2 (+ P1). Can not find Ge-donors. Interestingly, DJL two children’s red blood cells and DJL serum match. Then conducted a pedigree. RESULTS: Serology The following Ge phenotypes and genotypes were confirmed by untreated and trypsinized cells and erythrocyte membrane immunoblotting: DJL (Ge -): Ge: -2, -3,4 GYPC.Ge/GYPC. (Ge +): Ge: 2,3,4 GYPC / GYPC.Ge; Ge (Ge): Ge: 2,3,4 GYPC / GYPC.Yus; Ge: -2,3,4 GYPC.Ge/GYPC.Yus. The authors think: This study shows that there are two kinds in a family