Karpati G, Carpenter S, Prescott S
Muscle Nerve. 1982 May-Jun;5(5):369-72. doi: 10.1002/mus.880050506.
Necrosis and widespread central nucleation of skeletal muscle cells, which are the main features of skeletal muscle pathology in genetically dystrophic hamsters (UMX-7.1 strain), were not present in muscles of 45- to 65-day-old animals which had been surgically denervated at 15 to 18 days of age. Necrosis and widespread central nucleation were also absent in hind leg muscles at 30 to 60 days of age after transection of the high thoracic spinal cord at 15 to 18 days. Normal excitation or contraction of the skeletal muscle fibers in early age appears necessary for the microscopic pathological expression of the dystrophic gene in skeletal muscles in the UMX-7.1 strain hamsters. These experiments constitute a rare and clear example of consistent prevention of skeletal muscle cell destruction in vivo in an inherited myopathy.
坏死以及骨骼肌细胞广泛的中央核化是遗传性营养不良仓鼠(UMX - 7.1品系)骨骼肌病理学的主要特征,而在15至18日龄时接受手术去神经支配的45至65日龄动物的肌肉中并未出现这些特征。在15至18日龄时横断胸段高位脊髓后,30至60日龄的后腿肌肉中也未出现坏死和广泛的中央核化现象。在UMX - 7.1品系仓鼠中,早期骨骼肌纤维的正常兴奋或收缩对于营养不良基因在骨骼肌中的微观病理表达似乎是必要的。这些实验构成了一个罕见且明确的例子,即一致地在体内预防遗传性肌病中骨骼肌细胞的破坏。