Hardiman O, Sklar R M, Brown R H
Day Laboratory for Neuromuscular Research, Massachusetts General Hospital, Charlestown.
Neurology. 1993 Feb;43(2):342-5. doi: 10.1212/wnl.43.2.342.
Glucocorticoid therapy slows the progression of Duchenne muscular dystrophy. In muscle cultures, the addition of the glucocorticoid methylprednisolone increases myogenesis in most normal mixed and clonal cultures. Conversely, in some normal clonal and most dystrophic cultures, methylprednisolone inhibits fusion. However, in fusion-arrested normal and Becker muscular dystrophy cultures, dystrophin is expressed independently of fusion and of myosin heavy chain expression, and in some cases, expression is apparently enhanced by methylprednisolone. We suggest that dystrophin is a muscle-specific protein that does not require fusion for expression, and the methylprednisolone-induced enhancement of dystrophin expression may account for some of the clinical benefits of glucocorticoids in vivo.
糖皮质激素疗法可减缓杜氏肌营养不良症的进展。在肌肉培养物中，添加糖皮质激素甲泼尼龙可增加大多数正常混合培养物和克隆培养物中的肌生成。相反，在一些正常克隆培养物和大多数营养不良培养物中，甲泼尼龙会抑制融合。然而，在融合受阻的正常和贝克型肌营养不良培养物中，肌营养不良蛋白的表达独立于融合和肌球蛋白重链表达，并且在某些情况下，甲泼尼龙可明显增强其表达。我们认为，肌营养不良蛋白是一种肌肉特异性蛋白，其表达不需要融合，甲泼尼龙诱导的肌营养不良蛋白表达增强可能解释了糖皮质激素在体内的一些临床益处。
