Loirat M J, Pineau-Vincent F, Schiffer C, Muller J Y, Blanchard D
Centres Régionaux de Transfusion Sanguine, Nantes, France.
Vox Sang. 1996;70(2):92-6. doi: 10.1111/j.1423-0410.1996.tb01299.x.
The discovery of a natural Gerbich antigen (anti-Ge2) in the serum of a propositus prompted us to study his red blood cells (RBCs) by using monoclonal anti-bodies (mAbs) directed against glycophorin (GP) C and GPD. An mAb directed against the Ge4 antigen (mAb NaM10-7G11) agglutinated both untreated and trypsin-treated cells, demonstrating the expression of a trypsin-resistant GPC (namely, GPC of the Gerbich type: GPCGe). Surprisingly, an anti-Ge3 antibody (mAb NaM19-3C4) agglutinated untreated cells, showing that they also express the Ge3 antigen that may be carried by normal GPC and CPD or by the abnormal GPC of the Yussef (Yus) type (GPCYus). Immunoblotting analysis performed with an mAb directed against the C-terminal portion of GPC showed that the propositus' RBCs do not contain normal GPC and GPD but both GPCGe and GPCYus. Analysis of RBCs from the family demonstrated that, like the propositus, 2 of the 3 sisters had inherited both the GYPCGe and the GYPCYus alleles from the parents, who carried either the GYPCGe or the GYPCYus allele. The third sister had inherited the normal GYPC alleles from her parents, whereas the child of the propositus had inherited the GYPCGe allele. Interestingly, natural anti-Ge2 antibodies were identified in the serum of 2 of the 3 Ge-negative individuals.
在一名先证者的血清中发现天然的Gerbich抗原(抗-Ge2)后,我们使用针对血型糖蛋白(GP)C和GPD的单克隆抗体(mAb)对其红细胞(RBC)进行了研究。一种针对Ge4抗原的单克隆抗体(mAb NaM10 - 7G11)能凝集未处理和经胰蛋白酶处理的细胞,这表明存在一种对胰蛋白酶有抗性的GPC(即Gerbich型GPC:GPCGe)。令人惊讶的是,一种抗-Ge3抗体(mAb NaM19 - 3C4)能凝集未处理的细胞,这表明这些细胞也表达Ge3抗原,该抗原可能由正常的GPC和CPD携带,或者由Yussef(Yus)型异常GPC(GPCYus)携带。用针对GPC C末端部分的单克隆抗体进行的免疫印迹分析表明,先证者的红细胞不含正常的GPC和GPD,而是同时含有GPCGe和GPCYus。对该家族中红细胞的分析表明,与先证者一样,3个姐妹中有2个从父母那里继承了GYPCGe和GYPCYus等位基因,父母携带的是GYPCGe或GYPCYus等位基因。第三个姐妹从父母那里继承了正常的GYPC等位基因,而先证者的孩子继承了GYPCGe等位基因。有趣的是,在3个Ge阴性个体中的2个人的血清中发现了天然抗-Ge2抗体。