Fidziańska A, Glinka Z, Walski M
Department of Neurology, Medical School, Warszawa, Poland.
Clin Neuropathol. 1987 Nov-Dec;6(6):257-61.
The ultrastructural distribution of basement membrane around the capillaries as well as muscle cells of boys with Duchenne-type muscular dystrophy was determined. In dystrophic muscles, a diffuse thickness of vascular as well as muscular basement membrane was observed. Lamina densa lost its regular ribbon-like appearance and was split into several thin layers. After staining with tannic acid (TA), a densely stained meshwork was present on the muscle cell surface as well as in the extra-cellular space. Hyaluronidase treatment removed TA-stained deposits, indicating that hyaluronic acid is a major component. Enzyme resistant structures, presumably fibrous long-spacing collagen fibrils, have been found in dystrophic muscles. Based on the results of the study, faulty structure of the basement membrane in dystrophic muscle is suggested.
对患有杜兴氏型肌营养不良症男孩的毛细血管周围以及肌肉细胞的基底膜超微结构分布进行了测定。在营养不良的肌肉中，观察到血管以及肌肉基底膜弥漫性增厚。致密层失去了其规则的带状外观，分裂成几个薄层。用单宁酸（TA）染色后，在肌肉细胞表面以及细胞外空间出现了密集染色的网状结构。透明质酸酶处理去除了TA染色的沉积物，表明透明质酸是主要成分。在营养不良的肌肉中发现了抗酶结构，推测为纤维性长间距胶原纤维。基于该研究结果，提示营养不良肌肉中基底膜结构存在缺陷。
