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Animal models of muscular dystrophies

机译：肌肉营养不良的动物模型

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Recent advances in molecular biology have indicated that many mutant animal models of muscular dystrophy share common genetic and protein abnormalities similar to those of the human disease. The best example is a model of Duchenne muscular dystrophy (DMD), the mdx mouse. Similar to dystrophic muscle in DMD patients, dystrophic protein is not expressed along the surface membrane, even though the mdx mouse has no apparent signs of muscular dysfunction. Because clinical and pathologic findings in the dystrophic (mxd) dog are similar to those in DMD patients, it also has been regarded as a good model for therapeutic trials. The best known and most extensively studied dy+/dy+ mouse lacks merosin (laminin #alpha#2), which is one subunit of a basement membrane protein, laminin. Because approximately half of all patients with the classical form of congenital muscular dystrophy also lack merosin, availability of this animal has revived interest in the study of the pathologic mechanism of fiber necrosis resulting from this membrane defect. The dystrophic hamster is a model of limb-girdle muscular dystrophy with sarcoglycan deficiency in which one of the dystrophin-associated glycoproteins, #delta#-sarcoglycan, is defective. Because these animal models have common protein and genetic defects similar to those seen in people with muscular dystrophies, they have been widely used to examine the effectiveness of gene therapy and the administration of pharmacologic and trophic factors.
机译：分子生物学的最新进展表明，许多肌营养不良的突变动物模型具有与人类疾病相似的常见遗传和蛋白质异常。最好的例子是mdx小鼠杜兴氏肌营养不良症（DMD）的模型。与DMD患者的营养不良性肌肉相似，即使mdx小鼠没有明显的肌肉功能障碍迹象，营养不良蛋白也不会沿表面膜表达。由于营养不良（mxd）狗的临床和病理发现与DMD患者相似，因此也被认为是治疗试验的良好模型。最著名和最广泛研究的dy + / dy +小鼠缺少黑素（laminin＃alpha＃2），后者是基底膜蛋白层粘连蛋白的一个亚基。由于患有经典形式的先天性肌营养不良症的所有患者中大约有一半也缺乏黑素，因此该动物的可利用性引起了人们对由该膜缺损导致的纤维坏死的病理机制研究的兴趣。营养不良的仓鼠是伴有糖聚糖缺乏的四肢肌肉萎缩症的模型，其中与肌营养不良蛋白有关的糖蛋白之一，δ-肌糖蛋白是有缺陷的。由于这些动物模型具有常见的蛋白质和遗传缺陷，与肌肉营养不良的人相似，因此已被广泛用于检查基因治疗的有效性以及药理和营养因子的管理。

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