Bridges L R, Coulton G R, Howard G, Moss J, Mason R M
Department of Histopathology, Charing Cross and Westminster Medical School, London, United Kingdom.
Muscle Nerve. 1992 Feb;15(2):172-9. doi: 10.1002/mus.880150208.
We describe a new neuromuscular disorder in the kyphoscoliotic mouse mutant (ky). Mice were killed at ages from birth to 210 days, and tissues were taken for standard light microscopy, histochemistry, nerve ending studies, and electron microscopy. At birth a few myofibers showed phagocytosis ultrastructurally. Between 6 and 25 days there was prominent necrosis and regeneration in soleus, gracilis, paraspinal, and back muscles. At 47 days, these muscles were atrophic and necrosis and regeneration were rare. At 136 days, all muscle groups, including head muscles, showed some degree of myofiber atrophy and gracilis was fibrotic. Prominent intramuscular axonal sprouting was present from 31 days. Peripheral nerves and anterior horn cells were normal. The findings indicate a neuromuscular basis of hereditary kyphoscoliosis in the mouse. The animal may be useful as a model of human muscle disease and scoliosis.
我们描述了一种发生在脊柱后侧凸小鼠突变体（ky）中的新型神经肌肉疾病。在小鼠从出生到210天的不同年龄段将其处死，并取材进行标准光学显微镜检查、组织化学检查、神经末梢研究以及电子显微镜检查。出生时，少数肌纤维在超微结构上显示出吞噬现象。在6至25天之间，比目鱼肌、股薄肌、椎旁肌和背部肌肉出现明显的坏死和再生。在47天时，这些肌肉萎缩，坏死和再生现象罕见。在136天时，包括头部肌肉在内的所有肌肉组均显示出一定程度的肌纤维萎缩，股薄肌出现纤维化。从31天起出现明显的肌内轴突发芽。外周神经和前角细胞正常。这些发现表明小鼠遗传性脊柱后侧凸存在神经肌肉基础。该动物可能作为人类肌肉疾病和脊柱侧凸的模型。
