Wells D J, Wells K E
Department of Cellular and Molecular Neuroscience, Division of Neuroscience and Mental Health, Imperial College London, Charing Cross Hospital, UK.
Acta Myol. 2005 Dec;24(3):172-80.
Animal models of DMD have played, and will continue to play, a key role in the understanding of the pathogenesis and treatment of Duchenne muscular dystrophy (DMD). The mdx mouse and GRMD dog are spontaneous dystrophin deficient mutants and have been the most widely used models to date. A number of other murine models have been created by exposure to mutagens or genetic manipulation. The animal models have allowed the development of a number of promising experimental therapeutic approaches to DMD that are now entering clinical trial, the majority of which would not have been developed without their use. However, there has been much debate about the merits of the different animal models, which will only be finally clear as we learn from the initial human clinical trials.
杜氏肌营养不良症（DMD）的动物模型在理解杜氏肌营养不良症的发病机制和治疗方面已经发挥了并将继续发挥关键作用。mdx小鼠和GRMD犬是自发性肌营养不良蛋白缺陷突变体，是迄今为止使用最广泛的模型。通过暴露于诱变剂或基因操作已创建了许多其他小鼠模型。这些动物模型推动了多种有前景的DMD实验性治疗方法的发展，这些方法目前正在进入临床试验，其中大多数方法如果没有使用动物模型是不会被开发出来的。然而，关于不同动物模型的优点存在很多争论，只有当我们从最初的人体临床试验中吸取经验时，这一点才会最终明晰。
