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肌肉萎缩症及其他肌肉疾病中的肌纤维变性与坏死:细胞化学和免疫细胞化学数据

Muscle fiber degeneration and necrosis in muscular dystrophy and other muscle diseases: cytochemical and immunocytochemical data.

作者信息

Cornelio F, Dones I

出版信息

Ann Neurol. 1984 Dec;16(6):694-701. doi: 10.1002/ana.410160612.

Abstract

Serial consecutive frozen sections from 164 muscle biopsy specimens were studied cytochemically. We localized calcium and albumin as endogenous markers of extracellular fluid penetration and C3 and C9 complement components as markers of muscle fiber necrosis. In both Duchenne dystrophy and congenital muscular dystrophy, a significant percentage of fibers were overloaded with calcium and penetrated by albumin. Most of these fibers appeared opaque with trichrome stain. C3 and C9 complement components appeared only in necrotic fibers, which invariably were also penetrated by albumin. These observations support previous findings that muscle fiber necrosis is linked to massive inflow of extracellular fluid and complement activation. In addition, in both Duchenne and congenital dystrophies, numerous nonnecrotic fibers are penetrated by calcium-rich extracellular fluid.

摘要

对164份肌肉活检标本的连续冰冻切片进行了细胞化学研究。我们将钙和白蛋白定位为细胞外液渗透的内源性标志物,将C3和C9补体成分定位为肌纤维坏死的标志物。在杜兴肌营养不良症和先天性肌营养不良症中,相当比例的肌纤维钙超载且有白蛋白渗透。这些纤维中的大多数经三色染色后显得不透明。C3和C9补体成分仅出现在坏死纤维中,而这些坏死纤维也总是有白蛋白渗透。这些观察结果支持了先前的研究发现,即肌纤维坏死与细胞外液的大量流入和补体激活有关。此外,在杜兴肌营养不良症和先天性肌营养不良症中,许多非坏死纤维都有富含钙的细胞外液渗透。

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