Synthesis of aberrant decay-accelerating factor proteins by affected paroxysmal nocturnal hemoglobinuria leukocytes.
Synthesis of aberrant decay-accelerating factor proteins by affected paroxysmal nocturnal hemoglobinuria leukocytes.
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受影响的阵发性睡眠性血红蛋白尿白细胞合成异常的衰变加速因子蛋白。
DOI：
10.1172/jci114432
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发表时间：
1990
期刊：
影响因子：
--
通讯作者：
Medof,ME
中科院分区：
文献类型：
--
作者：
Carothers,DJ;Hazra,SV;Andreson,SW;Medof,ME
Paroxysmal nocturnal hemoglobinuria (PNH) leukocytes fail to express decay-accelerating factor (DAF) but contain DAF mRNA transcripts resembling those in normal cells. To further investigate the nature of the DAF defect in affected cells, patients' polymorphonuclear and mononuclear leukocytes (PMN and MNC) were biosynthetically labeled and newly synthesized DAF proteins examined. Analyses of greater than 98% surface DAF-negative PMN and MNC from a patient with PNH III erythrocytes showed precursor DAF protein approximately 3 kD smaller in each cell type than in normal cells. The proportion of precursor to mature (O-glycosylated) DAF protein was increased and soluble DAF protein was detected in the medium. Studies of 70-80% surface DAF-negative PMN and MNC from four patients with type II erythrocytes showed mixtures of the 3 kD smaller and normal DAF precursors. Partitioning with Triton X-114 detergent and biosynthetic labeling with the anchor precursor [3H]ethanolamine indicated that the abnormal peptides lacked glycosyl-inositolphospholipid membrane-anchoring structures. Thus, in PNH cells nascent DAF polypeptides are synthesized. Some of the abnormal pro-DAF molecules are processed in the Golgi and some are released extracellularly.Images
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DOI：
--
发表时间：
1988
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
Fatemi,SH;Tartakoff,AM
通讯作者：
Tartakoff,AM
人红细胞乙酰胆碱酯酶 C 末端糖脂膜结合域中胺成分的鉴定。
影响因子：
2.9
作者：
Haas,R;Brandt,PT;Knight,J;Rosenberry,TL
通讯作者：
Rosenberry,TL
阵发性睡眠性血红蛋白尿细胞中腐烂加速因子基因的正常多态性变异和转录。
DOI：
10.1073/pnas.85.3.880
发表时间：
1988
影响因子：
11.1
作者：
Stafford,HA;Tykocinski,ML;Lublin,DM;Holers,VM;Rosse,WF;Atkinson,JP;Medof,ME
通讯作者：
Medof,ME
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
人类补体调节蛋白衰变加速因子的生物合成和糖基化。
影响因子：
4.4
作者：
D. Lublin;J. Krsek;M. K. Pangburn;J. Atkinson
通讯作者：
J. Atkinson