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使用自旋标记法检测卵磷脂胆固醇酰基转移酶缺乏症患者红细胞膜的结构异常

Detection of erythrocyte membrane structural abnormalities in lecithin: cholesterol acyltransferase deficiency using a spin label approach.

作者信息

Maraviglia B, Herring F G, Weeks G, Godin D V

出版信息

J Supramol Struct. 1979;11(1):1-7. doi: 10.1002/jss.400110102.

Abstract

The membrane fluidity of erythrocytes from patients with Lecithin: cholesterol acyltransferase (LCAT) deficiency was studied by means of electron spin resonance. The temperature dependence of the separation of the outer extrema of the spectra of 2-(3-carboxy-propyl)-4,4-dimethyl, 2-tridecyl-3-oxazolidinyloxyl spin probe was monitored for normal, presumed carrier and clinically affected subjects. The temperature profile of controls was significantly different from that of the presumed carriers and the clinically affected individuals. The results show that the compositional abnormalities previously noted in erythrocyte membranes from patients with LCAT deficiency are associated with alterations in the physiocochemical state of the membrane. An investigation of the spectral lineshapes below 10 degrees C allowed a distinction to be made at the membrane level between clinically affected subjects and clinically normal heterozygous carriers. Alterations in the temperature dependence of elec-ron spin resonance parameters may provide a sensitive index of red cell membrane alterations in pathological states of generalized membrane involvement.

摘要

通过电子自旋共振研究了卵磷脂

胆固醇酰基转移酶(LCAT)缺乏症患者红细胞的膜流动性。监测了正常、推测携带者和临床受累受试者的2-(3-羧基丙基)-4,4-二甲基、2-十三烷基-3-恶唑烷氧基自旋探针光谱外极值分离的温度依赖性。对照组的温度曲线与推测携带者和临床受累个体的温度曲线有显著差异。结果表明,先前在LCAT缺乏症患者红细胞膜中发现的成分异常与膜的物理化学状态改变有关。对低于10摄氏度的光谱线形进行研究,使得在膜水平上能够区分临床受累受试者和临床正常的杂合子携带者。电子自旋共振参数温度依赖性的改变可能为广泛膜受累病理状态下红细胞膜改变提供一个敏感指标。

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