Electron spin resonance studies of erythrocytes from patients with Duchenne muscular dystrophy.

Electron spin resonance studies of erythrocytes from patients with Duchenne muscular dystrophy.
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杜氏肌营养不良症患者红细胞的电子自旋共振研究。

DOI:
10.1172/jci108934
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发表时间:
1978
期刊:
The Journal of clinical investigation
影响因子:
--
通讯作者:
F. H. Tyler
F. H. Tyler
中科院分区:
--
文献类型:
--
作者:
B. Sato;K. Nishikida;L. Samuels;F. H. Tyler

文献摘要

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用电子自旋共振方法研究了杜氏肌营养不良患者红细胞的膜组织。杜氏肌营养不良红细胞极区附近细胞膜的流动性与正常红细胞相似。以2-(14-羧基十四烷基)- 2-乙基- 4,4 -二甲基- 3 -恶唑烷氧基为探针的光谱判断,非极性区细胞膜环境与正常红细胞有很大不同。正常红细胞在pH 7.4时(分别为13.5°-16.5°和37.5°-40.5°)出现了两个拐点,而杜氏肌营养不良红细胞在较低温度范围内未发现拐点。当pH值变化时,正常红细胞在pH值为5.9 ~ 5.6时比值突然下降,而杜氏肌营养不良红细胞在pH值为6.6 ~ 5.0时比值逐渐下降。抗坏血酸对正常红细胞2-(3-羧基丙基)-4,4-二甲基-2-三环-3-恶唑烷氧基自由基的还原速率比杜氏肌营养不良红细胞快。用根皮素治疗两种红细胞可显著降低抗坏血酸的还原速率,消除两种红细胞的差异。这些结果表明,杜氏肌营养不良不仅累及肌细胞,还累及红细胞膜。
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.