Whitaker J N, Bertorini T E, Mendell J R
Ann Neurol. 1983 Feb;13(2):133-42. doi: 10.1002/ana.410130205.
The distribution of cathepsin D, an acidic endopeptidase, was localized by immunocytochemistry in human skeletal muscle obtained from 34 persons with a variety of neuromuscular disorders. Normal human skeletal muscle contained small amounts of cathepsin D, all of which was found close to the sarcolemmal membrane. Immunoreactive cathepsin D was present in the cytoplasm of many infiltrating phagocytic cells and was increased in skeletal muscle fibers from patients with muscular dystrophies, inflammatory myopathies, rhabdomyolysis, acid maltase deficiency, and neurogenic atrophy. In cases of Duchenne type muscular dystrophy, the increase in cathepsin D was especially prominent in small regenerating fibers, in which it was visualized at the ultrastructural level in lysosome-like organelles and extralysosomal locations. The function of cathepsin D in skeletal muscle is unclear, but the present findings suggest a possible role in muscle regeneration and repair. Such a role would necessitate careful selection of drugs which interfere with proteolytic activity if they are to be used as therapeutic agents in treating neuromuscular diseases.
通过免疫细胞化学方法，对34例患有各种神经肌肉疾病患者的人体骨骼肌中酸性内肽酶组织蛋白酶D的分布进行了定位。正常人体骨骼肌含有少量组织蛋白酶D，均位于肌膜附近。免疫反应性组织蛋白酶D存在于许多浸润性吞噬细胞的细胞质中，在患有肌营养不良症、炎性肌病、横纹肌溶解症、酸性麦芽糖酶缺乏症和神经源性萎缩的患者的骨骼肌纤维中含量增加。在杜兴型肌营养不良症病例中，组织蛋白酶D的增加在小的再生纤维中尤为明显，在超微结构水平上，可在溶酶体样细胞器和溶酶体外位置观察到。组织蛋白酶D在骨骼肌中的功能尚不清楚，但目前的研究结果表明其在肌肉再生和修复中可能发挥作用。如果要将干扰蛋白水解活性的药物用作治疗神经肌肉疾病的治疗剂，就需要谨慎选择此类药物。
