The acetylcholinesterase defect in paroxysmal nocturnal hemoglobinuria: evidence that the enzyme is absent from the cell membrane
The acetylcholinesterase defect in paroxysmal nocturnal hemoglobinuria: evidence that the enzyme is absent from the cell membrane
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阵发性睡眠性血红蛋白尿症中的乙酰胆碱酯酶缺陷：细胞膜上不存在该酶的证据
DOI：
10.1182/blood.v66.4.940.bloodjournal664940
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发表时间：
1985
期刊：
影响因子：
20.3
通讯作者：
W. Rosse
中科院分区：
文献类型：
--
作者：
F. Chow;M. Telen;W. Rosse
阵发性睡眠性血红蛋白尿症（PNH）是一种骨髓增生异常疾病，其特征是红细胞对补体介导的溶解表现出异常增加的敏感性。补体敏感性PNH红细胞膜先前已被证明缺乏乙酰胆碱酯酶（AchE）活性，但这种缺陷的分子基础尚不清楚。我们已经使用了四种不同的表位的乙酰胆碱酯酶分子的单克隆抗体，以显示异常PNH红细胞未能结合这些抗体。此外，异常PNH红细胞不含这些抗体的免疫沉淀蛋白，而正常补体不敏感的PNH患者的红细胞表现出正常量的免疫沉淀乙酰胆碱酯酶具有正常的电泳迁移率。这些数据表明，异常PNH红细胞缺乏AchE酶活性，由于缺乏AchE分子从细胞膜。
Paroxysmal nocturnal hemoglobinuria (PNH) is a myelodysplastic disease characterized by erythrocytes that show abnormally increased sensitivity to complement-mediated lysis. Complement-sensitive PNH erythrocyte membranes have previously been shown to lack acetylcholinesterase (AchE) activity, but the molecular basis of this deficiency has been unclear. We have used monoclonal antibodies to four different epitopes on the AchE molecule to show that abnormal PNH erythrocytes failed to bind these antibodies. Moreover, abnormal PNH erythrocytes contained no protein immunoprecipitable by these antibodies, while normal complement-insensitive erythrocytes from PNH patients showed normal amounts of immunoprecipitable AchE which had normal electrophoretic mobility. These data suggest that abnormal PNH erythrocytes lack AchE enzyme activity due to the absence of the AchE molecule from the cell membrane.
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人红细胞乙酰胆碱酯酶是一种两亲性蛋白质，其短膜结合域可通过木瓜蛋白酶消化去除。
DOI：
--
发表时间：
1984
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
Dutta-Choudhury,TA;Rosenberry,TL
通讯作者：
Rosenberry,TL
DOI：
10.1073/pnas.80.17.5430
发表时间：
1983-01-01
期刊：
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES
影响因子：
--
作者：
PANGBURN, MK;SCHREIBER, RD;MULLEREBERHARD, HJ
通讯作者：
MULLEREBERHARD, HJ
影响因子：
20.3
作者：
Telen,MJ;Palker,TJ;Haynes,BF
通讯作者：
Haynes,BF
DOI：
10.1073/pnas.80.16.5066
发表时间：
1983-01-01
期刊：
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES
影响因子：
--
作者：
NICHOLSONWELLER, A;MARCH, JP;AUSTEN, KF
通讯作者：
AUSTEN, KF