Muscular Dystrophy in a Dog Resembling Human Becker Muscular Dystrophy
Muscular Dystrophy in a Dog Resembling Human Becker Muscular Dystrophy
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DOI：
10.1016/j.jcpa.2013.12.006
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发表时间：
2014-05-01
影响因子：
0.8
通讯作者：
Paciello, O.
中科院分区：
文献类型：
--
作者：
Baroncelli, A. B.;Abellonio, F.;Paciello, O.
一只3岁的雄性拉布拉多寻回犬表现出进行性运动不耐受、双侧肘部伸展、前肢僵硬、后肢屈曲和脊柱后凸的临床体征。肌肉组织的显微镜检查显示肌纤维大小的显著变化、肌肉被成熟脂肪组织替代以及肌纤维变性/再生，与肌营养不良症一致。肌营养不良蛋白的免疫组织化学检查显示，与特异性的杆域和肌营养不良蛋白的羧基末端的单克隆抗体的标记显着减少，而β-肌聚糖，γ-肌聚糖和β-肌营养不良蛋白聚糖的表达是正常的。免疫印迹显示约135 kDa的截短的肌营养不良蛋白。这些发现支持了先天性犬肌营养不良症的诊断，类似于人类的贝克尔肌营养不良症。（C）2014爱思唯尔有限公司保留所有权利。
A 3-year-old, male Labrador retriever dog was presented with clinical signs of progressive exercise intolerance, bilateral elbow extension, rigidity of the forelimbs, hindlimb flexion and kyphosis. Microscopical examination of muscle tissue showed marked variability in myofibre size, replacement of muscle with mature adipose tissue and degeneration/regeneration of muscle fibres, consistent with muscular dystrophy. Immunohistochemical examination for dystrophin showed markedly reduced labelling with monoclonal antibodies specific for the rod domain and the carboxy-terminal of dystrophin, while expression of beta-sarcoglycan, gamma-sarcoglycan and beta-dystroglycan was normal. Immunoblotting revealed a truncated dystrophin protein of approximately 135 kDa. These findings supported a diagnosis of congenital canine muscular dystrophy resembling Becker muscular dystrophy in man. (C) 2014 Elsevier Ltd. All rights reserved.