Richards R B, Passmore I K
Department of Agriculture, Baron-Hay Court, South Perth, Western Australia.
Acta Neuropathol. 1989;79(2):168-75. doi: 10.1007/BF00294375.
The initial ultrastructural changes in skeletal myofibers in ovine muscular dystrophy (MD) consisted of focal degeneration of myofibrils and the formation of Z-disc abnormalities, including nemaline rods, in adjacent sarcomeres. Peripheral and central sarcoplasmic masses, which occurred initially in large diameter fibers, contained a mixture of normal organelles and abnormal tubular and fibrillar formations. Vesiculate sarcolemmal nuclei with prominent nucleoli accumulated in central and subsarcolemmal locations in small clusters and short rows. Deformed individual nuclei were sometimes present within nuclear rows. Loss of the myofibrillar mass, increased density of small spherical nuclei, collections of fibrillar and tubular arrays, excessive folding of the sarcolemma and greatly reduced fiber diameter were seen in the end stage of the dystrophic process. Resting satellite cells were present at all stages of lesion development. The morphological progression of the lesions suggested an inherited inability to effectively replace lost myofibrils with ultimate exhaustion of the capacity for repair followed by pathological fiber atrophy.
绵羊肌肉萎缩症（MD）骨骼肌纤维的初始超微结构变化包括肌原纤维的局灶性变性以及相邻肌节中Z盘异常的形成，包括线状体。最初出现在大直径纤维中的周边和中央肌浆块含有正常细胞器以及异常管状和纤维状结构的混合物。具有明显核仁的泡状肌膜细胞核在中央和肌膜下位置以小簇和短排的形式聚集。有时在核排内会出现变形的单个核。在营养不良过程的末期，可以看到肌原纤维物质的丧失、小球形核密度增加、纤维状和管状阵列的聚集、肌膜过度折叠以及纤维直径大大减小。在病变发展的各个阶段都存在静息卫星细胞。病变的形态学进展表明，由于遗传性的无法有效替代丢失的肌原纤维，最终导致修复能力耗尽，随后出现病理性纤维萎缩。
