Cullen M J, Fulthorpe J J
J Neurol Sci. 1975 Feb;24(2):179-200. doi: 10.1016/0022-510x(75)90232-4.
A scheme is presented which divides the process of fibre breakdown in Duchenne muscular dystrophy into 5 sequential stages. In Stage 1 the fibre appears superficially normal although there are subtle changes in the relative volumes of the fibre components. In Stage 2 localised overcontraction of the myofibrils results in excessive stretching of the sarcomeres in other regions of the fibre. This continuing process results in the formation of contraction clumps in which the contractile filaments form an increasingly homogeneous mass. By the final stage the normal structural features of the fibre have completely disappeared and it is invaded by macrophages. It is suggested that the central process of myofibril clumping results from a localised inability of the sarcomeres to relax which implies a defect in the mitochondria, Z-line or sarcoplasmic reticulum. This is discussed in relation to other workers' findings of a decrease in the calcium-accumulating capacity of the sarcoplasmic reticulum in Duchenne muscular dystrophy.
本文提出了一种将杜氏肌营养不良症中纤维分解过程分为5个连续阶段的方案。在第1阶段，纤维表面看起来正常，尽管纤维成分的相对体积存在细微变化。在第2阶段，肌原纤维的局部过度收缩导致纤维其他区域的肌节过度拉伸。这个持续的过程导致形成收缩团块，其中收缩细丝形成越来越均匀的物质。到最后阶段，纤维的正常结构特征完全消失，巨噬细胞侵入。有人认为，肌原纤维聚集的核心过程是由于肌节局部无法放松，这意味着线粒体、Z线或肌浆网存在缺陷。这与其他研究人员关于杜氏肌营养不良症中肌浆网钙积累能力下降的发现相关进行了讨论。
