摘要
本文用ＡＥ－１单克隆抗体和细胞亲和层析技术，从阵发性睡眠性血红蛋白尿症（ｐａｒｏｘｙｓｍａｌｎｏｃｔｕｒｎａｌｈｅｍｏｇｌｏｂｉｎｕｒｉａ，ＰＮＨ）患者外周血混合红细胞群中分离得到有膜缺陷的ＰＮＨ病态红细胞──缺乏乙酰胆碱脂酶［ＡｃｈＥ（－）］ＰＮＨ红细胞，并首次对这类纯化的病态细胞做了多方面的观察，结果显示ＡｃｈＥ（－）ＰＮＨ红细胞对补体溶血敏感、细胞变形性下降、细胞自氧化损伤明显、细胞膜上与ＡｃｈＥ结合的肌醇磷脂缺陷。提示ＰＮＨ溶血机制与ＰＮＨ红细胞膜内在缺陷导致多种病态特征有关。
A cell-affinity chromatography with AE-1 monoclonal antibody wasemployed to separate acetylcholinesterase deficient(AchE(-))PNH erythrocyte which were then explored the abnormalities in many aspects to identify the defect of the PNHerythrocytes leading to hemolysis.It was found for the first time that AchE (-)PNHerythrocytes were very susceptible to the complement,much decresed in cell deformability,and liable to be injured during auto-oxidization of the cell.Moreover,defect of phospha-tidylinositol(PI)which would be linked to variable membrane proteins like AchE,wasdetected as well.In conclusion,it is suggested that mechanism of hemolysis in PNHmight be pertinent to multi-abnormalities of PNH erythrocytes, and the membranedefect of the cell may play a crucial role in the pathophysiology of PNH.
出处
《中国病理生理杂志》
CAS
CSCD
北大核心
1994年第4期425-428,共4页
Chinese Journal of Pathophysiology
基金
国家自然科学基金
博士学科点专项科研基金
关键词
血红蛋白尿
红细胞
溶血
Erythrocyte
Hemoglobinuria
Hemolysis
