The Inab phenotype: characterization of the membrane protein and complement regulatory defect.
The Inab phenotype: characterization of the membrane protein and complement regulatory defect.
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DOI：
10.1182/blood.v74.1.437.bloodjournal741437
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发表时间：
1989-07
期刊：
影响因子：
20.3
通讯作者：
M. Telen;A. Green
中科院分区：
文献类型：
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作者：
M. Telen;A. Green
最近的证据表明，克罗默相关的人类血型抗原存在于衰变加速因子（decay-accelerating factor，简称ERF）上，导致了红细胞ERF的表观无效表型（apparent null phenotype，简称Inab）的鉴定。本研究检测了其他磷脂酰肌醇（PI）锚定蛋白的Inab红细胞的表达，并表明，PI连接的膜蛋白乙酰胆碱酯酶（AchE）和淋巴细胞功能相关抗原-3（LFA-3）正常表达这些细胞。此外，对Inab RBC的补体敏感性的研究表明，它们是异常补体敏感的，具有C3转化酶活性下调的明显缺陷。因此，Inab表型似乎代表遗传性红细胞血红蛋白缺乏症的一个实例，其机制不同于阵发性睡眠性血红蛋白尿症（PNH），并且与临床上明显的溶血性疾病无关。
Recent demonstration that Cromer-related human blood group antigens reside on decay-accelerating factor (DAF) has led to identification of an apparent null phenotype (Inab) for erythrocyte DAF. This study examined expression of other phosphatidylinositol (PI)-anchored proteins by Inab erythrocytes and showed that the PI-linked membrane proteins acetylcholinesterase (AchE) and lymphocyte function-associated antigen-3 (LFA-3) are normally expressed by these cells. Furthermore, studies of the complement sensitivity of Inab RBCs demonstrated them to be abnormally complement sensitive, with an apparent defect in downregulation of C3 convertase activity. Thus, the Inab phenotype appears to represent an instance of hereditary erythrocyte DAF deficiency whose mechanism differs from that of paroxysmal nocturnal hemoglobinuria (PNH) and which is unassociated with clinically evident hemolytic disease.