患有抗EnaFS和抗EnaFR的芬兰籍En(a-)先证者：体外和体内特征
Finnish En(a-) propositus with anti-EnaFS and anti-EnaFR: in vitro and in vivo characteristics.
作者信息
Walker P S, Bergren M O, Busch M P, Carmody A M, Perkins H A
出版信息
Vox Sang. 1987;52(1-2):103-6. doi: 10.1111/j.1423-0410.1987.tb03001.x.
A third Finnish En(a-) individual (ERP) with alloanti-Ena has been identified. Subsequent family studies revealed that ERP is distantly related to the 2 previous Finnish En(a-) propositi. Serologically, ERP's erythrocytes were found to be M-N-'N'+S-s+U+En(a-)Wr(a-b-) and were sialic acid deficient. SDS-PAGE studies confirmed that the red cell membranes lacked the MN-sialoglycoprotein, glycophorin-A. ERP's serum contained an IgG antibody which demonstrated two separable specificities, a ficin-sensitive specificity (anti-EnFS) and a ficin-resistant specificity (anti-EnFR), which reacted by the indirect antiglobulin technique. The antibodies were probably pregnancy induced, as ERP had never been transfused. A 51Cr-labelled red cell survival study showed that the antibodies were capable of causing significant destruction of incompatible red cells.
已鉴定出第三例携带抗Ena同种抗体的芬兰En(a-)个体（ERP）。随后的家族研究表明，ERP与之前的两例芬兰En(a-)先证者关系较远。血清学检测发现，ERP的红细胞为M-N-'N'+S-s+U+En(a-)Wr(a-b-)，且缺乏唾液酸。SDS-PAGE研究证实红细胞膜缺乏MN-唾液糖蛋白即血型糖蛋白A。ERP的血清中含有一种IgG抗体，该抗体表现出两种可分离的特异性，一种是对胰蛋白酶敏感的特异性（抗EnFS），另一种是对胰蛋白酶抵抗的特异性（抗EnFR），通过间接抗球蛋白技术发生反应。这些抗体可能是由妊娠诱导产生的，因为ERP从未接受过输血。一项51Cr标记的红细胞存活研究表明，这些抗体能够导致不相容红细胞的显著破坏。
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