Maier F, Bornemann A
Institute of Brain Research, University of Tübingen, Germany.
Muscle Nerve. 1999 May;22(5):578-83. doi: 10.1002/(sici)1097-4598(199905)22:5<578::aid-mus5>3.0.co;2-t.
Satellite cells are responsible for the formation of postnatal muscle fibers. The number, mitotic activity, and differentiation potential of satellite cells and the muscle fiber diameter are tightly regulated events in normal muscle. The signal that induces satellite cells to stop proliferation once the determined muscle fiber size has been reached in normal growth is not known. The aim of the present study was to determine whether a correlation exists between satellite cell frequency and muscle fiber diameter in human muscle disease. Muscle biopsies from 7 cases of Duchenne muscular dystrophy (DMD), 8 other muscular dystrophies, 23 cases of inflammatory myopathy, and 22 cases of neurogenic atrophy were examined. The satellite cell number was elevated in DMD and neurogenic atrophy but not in other muscular dystrophies or inflammatory myopathies. Nevertheless, in all the diseased muscles, but not in normal controls, there was a significantly higher relative frequency of satellite cells with increasing fiber diameter. It has been shown before that satellite cells show ultrastructural and autoradiographic signs of activation and proliferation in myopathic and neurogenic conditions. We assume that we are dealing with activated, not quiescent, satellite cells in diseased muscle and that under these conditions the fiber diameter does not represent a stop signal for satellite cells to proliferate. The data suggest that not only the number of satellite cells matters in diseased muscle, as has been shown before, but that it is their behavior that influences, at least in part, progress and severity of muscle diseases.
卫星细胞负责出生后肌纤维的形成。在正常肌肉中,卫星细胞的数量、有丝分裂活性、分化潜能以及肌纤维直径都是受到严格调控的事件。在正常生长过程中,一旦达到确定的肌纤维大小,诱导卫星细胞停止增殖的信号尚不清楚。本研究的目的是确定在人类肌肉疾病中卫星细胞频率与肌纤维直径之间是否存在相关性。对7例杜兴氏肌营养不良症(DMD)、8例其他肌营养不良症、23例炎性肌病和22例神经源性萎缩患者的肌肉活检样本进行了检查。DMD和神经源性萎缩患者的卫星细胞数量增加,但其他肌营养不良症或炎性肌病患者的卫星细胞数量未增加。然而,在所有患病肌肉中,而非正常对照中,随着纤维直径增加,卫星细胞的相对频率显著更高。之前已经表明,在肌病和神经源性疾病状态下,卫星细胞表现出激活和增殖的超微结构及放射自显影迹象。我们假设在患病肌肉中我们处理的是活化的而非静止的卫星细胞,并且在这些条件下纤维直径并不代表卫星细胞增殖的停止信号。数据表明,如之前所显示的,在患病肌肉中不仅卫星细胞的数量很重要,而且至少在一定程度上是它们的行为影响了肌肉疾病的进展和严重程度。