Pöche H, Schulze H
J Neurol Sci. 1985 Oct;70(3):295-304. doi: 10.1016/0022-510x(85)90171-6.
Ribosomes and ribosomal subunits were extracted from cultured skin fibroblasts from patients with Duchenne muscular dystrophy (DMD). A poly(U)-directed polyphenylalanine synthesis system was used to test 80S ribosomes from DMD cells and normal controls as well as hybrid 80S couples of subunits from DMD cells and control cells. The activity of ribosomes extracted from the patients was 38-62% lower than that of normal controls. Of the 80S hybrid ribosomes, only those consisting of 40S subunits from DMD cells and 60S subunits from the control cells, showed a similar decrease in activity. That means that the defect is exclusively based on an alteration in the small ribosomal subunit.
从杜氏肌营养不良症（DMD）患者的培养皮肤成纤维细胞中提取核糖体和核糖体亚基。使用聚（U）指导的聚苯丙氨酸合成系统来测试DMD细胞和正常对照的80S核糖体，以及DMD细胞和对照细胞的亚基组成的杂交80S核糖体对。从患者中提取的核糖体活性比正常对照低38 - 62%。在80S杂交核糖体中，只有那些由DMD细胞的40S亚基和对照细胞的60S亚基组成的核糖体，活性有类似程度的降低。这意味着缺陷完全是基于小核糖体亚基的改变。
