Ionasescu V, Zellweger H, Ionasescu R, Lara-Braud C, Cancilla P A
Acta Neurol Scand. 1976 Sep;54(3):241-7. doi: 10.1111/j.1600-0404.1976.tb04800.x.
Muscle samples for cultures were obtained from the quadriceps by open biopsy under local anesthesia in five patients with early stage of Duchenne muscular dystrophy (DMD) and 10 controls. Primary cultures were grown in Eagle's Minimum Essential Medium (MEM) with 20 per cent fetal calf serum. After 4 weeks, cells were trypsinized, counted, subcultured for 5 days in MEM with 5 per cent horse serum and finally incubated for 4 h with (3H) leucine. Ttal protein synthesis showed a significant decrease (half of control values) only in muscle cultures from patients with DMD. Addition of calclium chloride alone or with A23187 ionophore normalized this defect in protein synthesis. By contrast, myosin heavy chain synthesis was measured and found normal in all paitents.
在局部麻醉下，通过开放活检从五名杜兴氏肌营养不良症（DMD）早期患者的股四头肌获取用于培养的肌肉样本，并选取10名对照者作为对照。原代培养物在含有20%胎牛血清的伊格尔氏最低必需培养基（MEM）中生长。4周后，用胰蛋白酶处理细胞，计数，然后在含有5%马血清的MEM中传代培养5天，最后用（3H）亮氨酸孵育4小时。总蛋白合成仅在DMD患者的肌肉培养物中显著降低（为对照值的一半）。单独添加氯化钙或与A23187离子载体一起添加可使这种蛋白质合成缺陷恢复正常。相比之下，对肌球蛋白重链合成进行了测量，发现所有患者的该合成均正常。
