Fanin M, Danieli G A, Cadaldini M, Miorin M, Vitiello L, Angelini C
Department of Neurology, University of Padova, Italy.
Muscle Nerve. 1995 Oct;18(10):1115-20. doi: 10.1002/mus.880181007.
In 132 DMD muscle biopsies we investigated the presence of dystrophin-positive fibers and the relationship of dystrophin immunohistochemical pattern to the clinical severity of the disease. Reverted fibers were detected in 37% of patients; their prevalence increased significantly in each biopsy with age of patients. We suggest that reversion occurs in satellite cells, when muscle differentiation is completed. The longitudinal extent of dystrophin-positive domain spans a maximum length of 900 microns. No correlation was found between the presence of reverted fibers and the clinical severity of DMD, whereas a milder form of Duchenne dystrophy was observed in patients showing a faint reaction in all fibers. The occurrence of reverted fibers is independent of the type of gene mutation; however, a higher proportion of cases with reverted fibers was found among patients with gene duplications.
在132份杜氏肌营养不良（DMD）肌肉活检样本中，我们研究了抗肌萎缩蛋白阳性纤维的存在情况以及抗肌萎缩蛋白免疫组化模式与疾病临床严重程度之间的关系。在37%的患者中检测到了回复纤维；随着患者年龄增长，每次活检中回复纤维的患病率显著增加。我们认为，当肌肉分化完成时，卫星细胞中会发生回复现象。抗肌萎缩蛋白阳性区域的纵向范围最大跨度为900微米。未发现回复纤维的存在与DMD临床严重程度之间存在相关性，而在所有纤维中反应微弱的患者中观察到了较轻形式的杜氏肌营养不良。回复纤维的出现与基因突变类型无关；然而，在基因重复的患者中发现回复纤维的病例比例更高。
