Duchenne型肌营养不良症肌细胞膜异常的研究

The study of Membrane Abnormalities in Duchenne Muscular Dystrophy

本文应用凝集素免疫电镜和冷冻断裂电镜技术、通过对６例ＤＭＤ患者组和４例健康对照组骨骼肌细胞膜的观察，发现ＲＣＡ－Ｉ和ＷＧＡ凝集素受体主要分布在肌质膜上，ＤＭＤ患者有４０％左右的肌质膜存在局限性的受体分布缺损区，并且ＤＭＤ肌质膜的ＰＦ面和ＥＦ面上的膜蛋白颗粒还明显减少，从分子病理学水平证实了ＤＭＤ存在肌细胞膜的异常。

Using lectin immunoelectronmicroscopy and freeze-fracture electron microscopy,we searched for differences in skeletal muscle membrare of 4 normal controls and 6 patients with DMD.Electron microscopy observation revealed many local absences of RCA- I and WGA lectin receptors in muscle plasma membrane in DMD. Intramembrane particles of both EF and PF faces are diminished in DMD as compared with those of normal control.The results provide good evidence that a basic structurall change within the plasma membrane may be associated with DMD.