Burr I M, Asayama K, Fenichel G M
Muscle Nerve. 1987 Feb;10(2):150-4. doi: 10.1002/mus.880100208.
Studies in experimental muscular dystrophy indicate a possible role for anomalous redox metabolism in the genesis of these disorders, prompting a retrospective review of changes in redox-active enzymes in Duchenne muscular dystrophy (DMD). Both manganous and copper-zinc superoxide dismutase (Mn and CuZn SOD) content and glutathione peroxidase and catalase activities were measured in muscle biopsy specimens taken from normal individuals and from patients with Duchenne muscular dystrophy and other neuromuscular diseases. Muscle from patients with Duchenne dystrophy differed from the norm in that both Mn SOD and CuZn SOD were decreased and glutathione peroxidase was increased. This profile differed from that in anterior horn cell diseases in that CuZn SOD was not decreased in these disorders and from polymyositis, where CuZn SOD was decreased without an increase in glutathione peroxidase. Thus, there appears to be disease-specific changes in these enzymes in DMD. These data support the concept that changes in redox-active enzymes may be associated with the genesis of DMD.
对实验性肌肉萎缩症的研究表明，异常氧化还原代谢在这些疾病的发生过程中可能发挥作用，这促使人们对杜兴氏肌肉萎缩症（DMD）中氧化还原活性酶的变化进行回顾性研究。对取自正常个体以及杜兴氏肌肉萎缩症患者和其他神经肌肉疾病患者的肌肉活检标本，测定了锰超氧化物歧化酶和铜锌超氧化物歧化酶（Mn和CuZn SOD）的含量以及谷胱甘肽过氧化物酶和过氧化氢酶的活性。杜兴氏肌肉萎缩症患者的肌肉与正常情况不同，表现为Mn SOD和CuZn SOD均降低，而谷胱甘肽过氧化物酶增加。这种情况与前角细胞疾病不同，在前角细胞疾病中CuZn SOD没有降低；也与多发性肌炎不同，在多发性肌炎中CuZn SOD降低但谷胱甘肽过氧化物酶没有增加。因此，在DMD中这些酶似乎存在疾病特异性变化。这些数据支持氧化还原活性酶的变化可能与DMD的发生有关这一概念。
