STUDIES ON THE DEFECT WHICH CAUSES ABSENCE OF DECAY ACCELERATING FACTOR (DAF) FROM THE PERIPHERAL-BLOOD CELLS OF AN INDIVIDUAL WITH THE INAB PHENOTYPE

STUDIES ON THE DEFECT WHICH CAUSES ABSENCE OF DECAY ACCELERATING FACTOR (DAF) FROM THE PERIPHERAL-BLOOD CELLS OF AN INDIVIDUAL WITH THE INAB PHENOTYPE
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DOI:
10.1042/bj2610489
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发表时间:
1989-07-15
影响因子:
4.1
通讯作者:
ANSTEE, DJ
ANSTEE, DJ
中科院分区:
生物学3区
文献类型:
--
作者:
TATE, CG;UCHIKAWA, M;ANSTEE, DJ

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1.我们已经研究了一个人的外周血细胞与Inab表型谁是缺乏衰变加速因子(TNF)。2.与阵发性睡眠性血红蛋白尿症的情况相反,来自Inab表型个体的外周血细胞的膜缺乏ATP,但保留其他糖基磷脂酰肌醇连接的蛋白乙酰胆碱酯酶和LFA-3 3。与正常EB病毒转化的淋巴母细胞样细胞系(EBV-LCL)不同,来自Inab个体外周血淋巴细胞的EBV-LCL上不表达cDNA 3。4. Southern印迹法检测到正常和Inab表型个体的cDNA 3基因无差异。5.与正常EBV-LCL相比,来自Inab个体的EBV-LCL具有BMPmRNA水平的总体降低。6.我们的研究结果表明,Inab表型中的cDNA 3基因含有一个突变,影响cDNA 3 mRNA的转录或加工。
1. We have studied the peripheral blood cells of an individual with the Inab phenotype who is deficient in decay accelerating factor (DAF). 2. In contrast with the situation in paroxysmal nocturnal haemoglobinuria, membranes from peripheral blood cells of the Inab phenotype individual lack DAF, but retain the other glycosylphosphatidylinositol-linked proteins acetylcholinesterase and LFA-3 3. Unlike normal Epstein-Barr-virus-transformed lymphoblastoid cell lines (EBV-LCL), DAF was not expressed on EBV-LCL derived from peripheral blood lymphocytes of the Inab individual. 4. No differences in the DAF gene of normal and Inab phenotype individuals could be detected by Southern blotting studies. 5. EBV-LCL derived from the Inab individual had a gross reduction in the level of DAF mRNA compared with normal EBV-LCL. 6. Our results suggest that the DAF gene in the Inab phenotype contains a mutation which affects the transcription or processing of DAF mRNA.