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恩(a-)红细胞膜糖蛋白缺陷的研究。II. 恩(a-)红细胞的MN抗原特性。

Studies on the membrane glycoprotein defect of En(a-) erythrocytes. II. MN antigenic properties of En(a-) erythrocytes.

作者信息

Dahr W, Uhlenbruck G, Wagstaff W, Leikola J

出版信息

J Immunogenet. 1976 Dec;3(6):383-93. doi: 10.1111/j.1744-313x.1976.tb00599.x.

Abstract

En(a-) and EnaEn red cells from different sources were studied using biochemical and serological methods. The results suggest that Finnish En(a-) erythrocytes contain only "N" but no M antigenic properties. Data on the members of the English En(a-) family suggest that English En(a-) red cells exhibit a combination of three rare alterations. The English En(a-) individuals are apparently heterozygous for the defects En and Ms, to all appearances, converted to a M ("M") antigen. These extraordinary data are brought to light by investigations on the children of the propositus (G.P. and J.P.), whose red cells are M-N+S-s+"N"+"M"- and M-N+S+s+"N"+"M"+ respectively. Sodium dodecylsulphate polyacrylamide gel electrophoretic results indicate that the MN glycoprotein content is decreased by about 50% in all English S+s+EnaEn red cells, the Ss glycoprotein being normal. G.P. erythrocytes, however, have only about half of the normal MN and Ss glycoprotein content.

摘要

使用生化和血清学方法研究了来自不同来源的En(a-)和EnaEn红细胞。结果表明,芬兰的En(a-)红细胞仅含有“N”,而没有M抗原特性。关于英国En(a-)家族成员的数据表明,英国的En(a-)红细胞表现出三种罕见改变的组合。英国的En(a-)个体显然是En和Ms缺陷的杂合子,表面上转变为M(“M”)抗原。对先证者(G.P.和J.P.)子女的研究揭示了这些非凡的数据,其红细胞分别为M-N+S-s+“N”+“M”-和M-N+S+s+“N”+“M”+。十二烷基硫酸钠聚丙烯酰胺凝胶电泳结果表明,所有英国S+s+EnaEn红细胞中的MN糖蛋白含量降低了约50%,而Ss糖蛋白正常。然而,G.P.的红细胞中MN和Ss糖蛋白含量仅约为正常水平的一半。

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