Sato B, Nishikida K, Samuels L T, Tyler F H
J Clin Invest. 1978 Feb;61(2):251-9. doi: 10.1172/JCI108934.
The membrane organization of the erythrocytes from patients with Duchenne muscular dystrophy was studied by means of electron spin resonance. The fluidity of the membrane near the polar region of Duchenne muscular dystrophy erythrocytes was similar to that of normal erythrocytes. The membrane environment in the nonpolar region, however, was quite different from that of normal erythrocytes, judged by the spectra with 2-(14-carboxytetradecyl) - 2 - ethyl - 4,4 - dimethyl - 3 - oxazolidinyloxyl as probe. The temperature dependence of the ratio of the line height of central field to that at the low field showed two inflection points in normal erythrocytes at pH 7.4 (13.5 degrees -16.5 degrees and 37.5 degrees -40.5 degrees C, respectively) but the inflection point in the lower temperature range was not detected in Duchenne muscular dystrophy erythrocytes. When pH was varied, an abrupt decrease in the ratio was observed at pH 5.9-5.6 in normal erythrocytes whereas there was a gradual decrease over the range of pH from 6.6 to 5.0 in Duchenne muscular dystrophy erythrocytes. The rate of reduction of the radical 2-(3-carboxypropyl)-4,4-dimethyl-2-tridecyl-3-oxazolidinyloxyl by ascorbate in normal erythrocytes was faster than that in Duchenne muscular dystrophy erythrocytes. Treatment of both erythrocytes with phloretin markedly reduced the rate of reduction by ascorbate and eliminated the difference in the two types of erythrocyte. These results indicate that in Duchenne muscular dystrophy the erythrocyte membrane is involved as well as the muscle cell.
采用电子自旋共振方法研究了杜兴氏肌营养不良症患者红细胞的膜结构。杜兴氏肌营养不良症红细胞极性区域附近膜的流动性与正常红细胞相似。然而,以2-(14-羧基十四烷基)-2-乙基-4,4-二甲基-3-恶唑烷氧基为探针,通过光谱判断,非极性区域的膜环境与正常红细胞有很大不同。在pH 7.4时,正常红细胞中心场与低场线高比的温度依赖性显示出两个拐点(分别为13.5℃-16.5℃和37.5℃-40.5℃),但杜兴氏肌营养不良症红细胞未检测到低温范围内的拐点。当pH值变化时,正常红细胞在pH 5.9-5.6时该比值突然下降,而杜兴氏肌营养不良症红细胞在pH值从6.6到5.0的范围内逐渐下降。正常红细胞中抗坏血酸对自由基2-(3-羧基丙基)-4,4-二甲基-2-十三烷基-3-恶唑烷氧基的还原速率比杜兴氏肌营养不良症红细胞快。用根皮素处理两种红细胞均显著降低了抗坏血酸的还原速率,并消除了两种红细胞之间的差异。这些结果表明,在杜兴氏肌营养不良症中,红细胞膜与肌肉细胞一样受到影响。