Kumamoto T, Ueyama H, Sugihara R, Kominami E, Goll D E, Tsuda T
Third Department of Internal Medicine, Oita Medical University, Japan.
Eur Neurol. 1997;37(3):176-81. doi: 10.1159/000117430.
To clarify the significance of intracellular lysosomal (cathepsins B, L and H) and extralysosomal (calpain) proteolytic systems in the process of muscle fiber degradation in inflammatory myopathies, biopsied muscle specimens were examined from patients with polymyositis (PM) and dermatomyositis (DM). Generally, in specimens from patients with PM and DM, but not in those from normal controls, muscle fibers surrounding inflammatory infiltrates or in the perifascicular regions, and occasionally mononuclear cell infiltrates demonstrated positive immunostaining for calpain and cathepsins B, L, and H. In addition, enzyme activities of cathepsins B and L increased in specimens with inflammatory myopathy. These results suggest that calpain and cathepsins play a significant role in the process of muscle fiber destruction in inflammatory myopathy.
为阐明细胞内溶酶体（组织蛋白酶B、L和H）和细胞外溶酶体（钙蛋白酶）蛋白水解系统在炎性肌病中肌纤维降解过程中的意义，对多肌炎（PM）和皮肌炎（DM）患者的活检肌肉标本进行了检查。一般来说，在PM和DM患者的标本中，而非正常对照者的标本中，炎症浸润周围或束周区域的肌纤维，以及偶尔的单核细胞浸润对钙蛋白酶和组织蛋白酶B、L和H呈阳性免疫染色。此外，炎性肌病标本中组织蛋白酶B和L的酶活性增加。这些结果表明，钙蛋白酶和组织蛋白酶在炎性肌病的肌纤维破坏过程中起重要作用。
