Weller A H, Magliato S A, Bell K P, Rosenberg N L
Department of Neuroimmunology, Colorado Neurological Institute, Englewood, USA.
Muscle Nerve. 1997 Jan;20(1):72-82. doi: 10.1002/(sici)1097-4598(199701)20:1<72::aid-mus10>3.0.co;2-3.
Myopathy has been found to develop spontaneously in 100% of SJL/J mice between 6 and 8 months of age. Extent of muscular involvement and mouse strength were quantified in SJL/J mice and Balb/c control mice 2-16 months old. Muscle from young SJL/J mice exhibited histopathological abnormalities and occasional inflammatory infiltrate. By 6 months, 78% of SJL/J mice had developed active myopathy. By 8 months, all SJL/J mice examined had active disease with a mean of 12.9% of muscle fibers affected. Replacement of muscle fibers by fat and/or collagen began at 10 months and was pronounced by 14 months. Significant decreases in strength scores (total body pulling force) at 6 months and 10 months of age reflected the onset of active myopathy and the onset of muscle degeneration, respectively. The spontaneous onset and 100% incidence of myopathy in the SJL/J mouse line should provide a useful model for idiopathic myopathy.
已发现100%的SJL/J小鼠在6至8月龄时会自发发生肌病。对2至16月龄的SJL/J小鼠和Balb/c对照小鼠的肌肉受累程度和小鼠力量进行了量化。年轻SJL/J小鼠的肌肉表现出组织病理学异常和偶尔的炎性浸润。到6个月时，78%的SJL/J小鼠发生了活动性肌病。到8个月时，所有接受检查的SJL/J小鼠均患有活动性疾病，平均有12.9%的肌纤维受到影响。脂肪和/或胶原对肌纤维的替代在10个月时开始，到14个月时明显。6月龄和10月龄时力量评分（全身拉力）的显著降低分别反映了活动性肌病的发作和肌肉变性的发作。SJL/J小鼠品系中肌病的自发发作和100%的发病率应为特发性肌病提供一个有用的模型。
