Animal Models of Duchenne Muscular Dystrophy, with Special Reference to the mdx Mouse

作者：Blain Alison^*; Greally Elizabeth; Laval Steve; Blamire Andrew; MacGowan Guy; Straub Volker

来源：Biocybernetics and Biomedical Engineering, 2012, 32(4): 3-15.

• duchenne muscular dystrophy
• mdx mouse
• cardiomyopathy
• animal models
• MRI
• cardiac catheter
• standard operating procedures

Duchenne muscular dystrophy (DMD) is a progressive muscle wasting disease that affects approximately 1 in 3500 male births. We describe animal models of DMD with special reference to the mdx mouse. We also describe some of the standard operating procedures (SOPs) developed by the TREAT-NMD neuromuscular network (http://www.treat-nmd.eu/) for assessment of the mdx mouse, with a focus on techniques for assessing cardiac function that are used in our lab, including the cardiac conductance catheter. We have also recently developed cardiac MRI as a novel cardiac assessment technique for mouse models of muscular dystrophy. We describe how this technique can be used both in the assessment of ventricular function and in the investigation of the role of abnormal calcium influx in muscular dystrophy-associated cardiomyopathy.

• 出版日期2012
• 单位河北医科大学