Deficiency of the homologous restriction factor in paroxysmal nocturnal hemoglobinuria.
Deficiency of the homologous restriction factor in paroxysmal nocturnal hemoglobinuria.
复制标题
阵发性夜间血红蛋白尿中的同源限制因子的缺乏。
DOI：
10.1084/jem.165.2.572
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发表时间：
1987-02-01
影响因子：
15.3
通讯作者：
MULLEREBERHARD, HJ
中科院分区：
文献类型：
--
作者：
ZALMAN, LS;WOOD, LM;FRANK, MM;MULLEREBERHARD, HJ
本文用抗人衰变加速因子（decayacceleratingfactor，简称ERF）和豚鼠血清裂解正常E，富集了2例阵发性睡眠性血红蛋白尿症（PNH）患者的E。溶解未溶解的DAF缺陷细胞（PNH-E）的膜，并使用同源限制因子（HRF）的抗血清通过SDS-PAGE和免疫印迹进行检查。而65 kD补体调节蛋白在正常对照中很容易检测到，它是完全缺乏的两个样品的PNH-E膜。功能研究同样表明PNH-E中不存在HRF活性。当将放射性标记的分离的HRF蛋白提供给PNH-E时，它牢固地附着在细胞上。每个细胞大约1,000个HRF分子将这些细胞对C5b-9反应性裂解的特征敏感性降低到接近正常水平。结果表明，已知控制C8和C9对正常人E膜的作用的HRF缺乏PNH，以及乙酰胆碱酯酶和乙酰胆碱酯酶。
The affected E of two patients with paroxysmal nocturnal hemoglobinuria (PNH) were enriched by lysing the unaffected, normal E with anti-human decay-accelerating factor (DAF) and guinea pig serum. The membranes of the unlysed, DAF-deficient cells (PNH-E) were dissolved and examined by SDS-PAGE and immunoblotting using an antiserum to homologous restriction factor (HRF). Whereas the 65 kD complement regulatory protein was readily detectable in the normal controls, it was completely lacking in both samples of PNH-E membranes. Functional studies likewise indicated the absence of HRF activity from PNH-E. When radiolabeled, isolated HRF protein was offered to PNH-E, it became firmly attached to the cell. Approximately 1,000 molecules of HRF per cell reduced the characteristic susceptibility of these cells to reactive lysis by C5b-9 to nearly normal levels. The results suggest that HRF, which is known to control the action of C8 and C9 on normal human E membranes, is deficient in PNH, as well as acetylcholinesterase and DAF.
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DOI：
10.1073/pnas.82.9.2980
发表时间：
1985-01-01
影响因子：
11.1
作者：
MEDOF, ME;KINOSHITA, T;NUSSENZWEIG, V
通讯作者：
NUSSENZWEIG, V
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
DOI：
10.1084/jem.157.6.1971
发表时间：
1983-06-01
期刊：
The Journal of experimental medicine
影响因子：
--
作者：
Pangburn MK;Schreiber RD;Trombold JS;Müller-Eberhard HJ
通讯作者：
Müller-Eberhard HJ
DOI：
10.1002/path.1700550318
发表时间：
1943-07-01
期刊：
JOURNAL OF PATHOLOGY AND BACTERIOLOGY
影响因子：
--
作者：
Dacie, JV;Richardson, N
通讯作者：
Richardson, N
正常个体和阵发性夜间血红蛋白尿症患者外周血中衰减加速因子的分布。
影响因子：
15.3
作者：
Kinoshita, T;Medof, M E;Silber, R;Nussenzweig, V
通讯作者：
Nussenzweig, V