【摘要】 <正> 阵发性睡眠性血红蛋白尿症（PNH）是一种红细胞膜异常的溶血性疾病,其特征之一是对补体敏感,但机理不明。Rosse[1]观察,PNH病人红细胞膜结合补体3b(C_3b)的数量比正常人多3～7倍。Musscl[2]最近指出人红细胞C_3b受体是糖蛋白,并证实C_3b与其受体结合时糖基起主要作用。唾液酸常位于糖蛋白寡糖链的末端,可推测当两者结合时唾液酸是不可缺少的。所以我们对正常人与PNH病人红细胞膜上唾液酸与补体溶血的关系进行研究,以期对PNH发病机理有进一步了解。更多还原

【Abstract】 Erythrocytes were treated with neuramidase to remove sialic acid and with horseshoe crab blood lectin to block sialic acid. The complement mediated hemolysis of normal and PNH erythrocytes was measured by cobra venom factor hcmolysis test. After neuramidase treatment, normal erythrocytes showed no significant change in hemolysis, while erythrocytes from 6 out of 8 PNH patients hemolysed to a lesser extent after such a treatment. Hemolysis of normal erythrocytes was only sightly decreased if preincubated with horseshoe crab blood lectin, while erythrocytes of 4 of 8 PNH patients so treated showed remarkable declination in hemolysis. These proved that sialic acid is essential in complement mediated hemolysis and different PNH erythrocytes differ in their response to the modification of surface sialic acid, probably in accordance with their original sensaivity to complement.更多还原