Marshall P A, Williams P E, Goldspink G
University of Hull, Department of Zoology, UK.
Muscle Nerve. 1989 Jul;12(7):528-37. doi: 10.1002/mus.880120703.
The growth and development of the X-linked muscular dystrophy mutant mouse (mdx) was compared with a control group from 3 weeks to 1 year old. Quantitative cytological analysis of the soleus muscle revealed cycles of degeneration, regeneration, and hypertrophy, and at any one time it was difficult to assess the extent of the disease based on muscle fiber size. One noticeable difference even in the youngest muscles studied was the reduced numbers of slow oxidative fibers and the increased number of fast glycolytic fibers in the mdx soleus muscles. The collagen of the connective tissue components of selectively stained sections was determined by computerized image analysis. Marked accumulation of collagen was found in both the endomysium and perimysium of the dystrophic muscles as compared with age-matched controls. Since the mdx mouse is a result of the same type of genetic defect as in human Duchenne muscular dystrophy, this model could thus be used to assess the effectiveness of various therapeutic approaches, including gene therapy using muscle fibrosis and fiber type proportions as the indicators.
将X连锁型肌营养不良突变小鼠（mdx）与对照组从3周龄至1岁进行生长发育比较。对比目鱼肌进行定量细胞学分析，发现其存在退化、再生和肥大的循环，而且在任何一个时间点，都很难基于肌纤维大小来评估疾病的程度。即使在研究的最年幼小鼠的肌肉中，一个明显的差异是mdx比目鱼肌中慢氧化纤维数量减少，快糖酵解纤维数量增加。通过计算机图像分析确定选择性染色切片结缔组织成分中的胶原蛋白。与年龄匹配的对照组相比，在营养不良肌肉的肌内膜和肌束膜中均发现明显的胶原蛋白积累。由于mdx小鼠是由与人类杜氏肌营养不良相同类型的基因缺陷导致的，因此该模型可用于评估各种治疗方法的有效性，包括以肌肉纤维化和纤维类型比例为指标的基因治疗。
