Gaffney B J, Drachman D B, Lin D C, Tennekoon G
Neurology. 1980 Mar;30(3):272-6. doi: 10.1212/wnl.30.3.272.
The basic defect in myotonic dystrophy is thought to involve muscle cell membranes. Butterfield and associates have recently presented electron spin resonance data that suggest increased fluidity of erythrocyte membranes in patients with myotonic dystrophy. We studied erythrocytes from 11 patients with myotonic dystrophy and 14 age-matched controls, using spin-labeled fatty acid and ester probes. Despite attempts to reproduce the previously reported experimental conditions exactly, we found no significant differences in the electron spin resonance spectra of erythrocytes from normal and myotonic dystrophy subjects. These findings do not provide evidence of increased erythrocyte membrane fluidity in myotonic dystrophy; they fail to support the concept of an intrinsic defect of the lipid membrane in this disorder.
强直性肌营养不良的基本缺陷被认为与肌肉细胞膜有关。巴特菲尔德及其同事最近发表的电子自旋共振数据表明，强直性肌营养不良患者的红细胞膜流动性增加。我们使用自旋标记的脂肪酸和酯探针，研究了11例强直性肌营养不良患者和14例年龄匹配的对照者的红细胞。尽管试图精确重现先前报道的实验条件，但我们发现正常人和强直性肌营养不良患者的红细胞电子自旋共振光谱没有显著差异。这些发现没有提供强直性肌营养不良患者红细胞膜流动性增加的证据；它们不支持这种疾病中脂膜存在内在缺陷的概念。
