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肌萎缩侧索硬化症中细胞毒性血浆活性的性质与特性

Nature and properties of cytotoxic plasma activity in amyotrophic lateral sclerosis.

作者信息

Ronnevi L O, Conradi S, Karlsson E, Sindhupak R

机构信息

Department of Clinical Neurology, Karolinska sjukhuset, Stockholm, Sweden.

出版信息

Muscle Nerve. 1987 Oct;10(8):734-43. doi: 10.1002/mus.880100810.

DOI:10.1002/mus.880100810
PMID:3683447
Abstract

Cytotoxic activity of plasma towards normal red blood cells in patients with amyotrophic lateral sclerosis (ALS) has been studied as a function of progressive plasma dilution and compared with plasma from patients with Charcot-Marie-Tooth's disease (CMT). At progressive dilution the hemolysis by ALS-plasma showed a specific pattern that differed qualitatively and quantitatively from that of normal plasma as well as CMT and persisted up to a dilution of 1:6561. Differences in dilution pattern were found when comparing different clinical types of ALS. There was evidence for a partial complement dependency of the reaction that brings about the hemolysis provoked by ALS plasma. Experiments with plasma fractionated by gel filtration and with isolated immunoglobulins produced evidence for cytotoxic properties of IgA and IgG from ALS plasma. The observations speak in favor of a consistency between the observations of plasma cytotoxicity in ALS and earlier observations on immunological abnormalities in the disease.

摘要

对肌萎缩侧索硬化症(ALS)患者血浆对正常红细胞的细胞毒性活性进行了研究,该活性是血浆逐步稀释的函数,并与夏科-马里-图思病(CMT)患者的血浆进行了比较。在逐步稀释过程中,ALS患者血浆的溶血表现出一种特定模式,在质量和数量上均不同于正常血浆以及CMT患者血浆,并且这种差异一直持续到1:6561的稀释度。比较不同临床类型的ALS时,发现了稀释模式的差异。有证据表明,导致ALS血浆引起溶血的反应部分依赖补体。通过凝胶过滤分离血浆以及使用分离的免疫球蛋白进行的实验证明了ALS血浆中IgA和IgG的细胞毒性特性。这些观察结果支持了ALS患者血浆细胞毒性的观察结果与该疾病早期免疫异常观察结果之间的一致性。

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