Delaporte C, Dehaupas M, Fardeau M
J Neurol Sci. 1984 May;64(2):149-60. doi: 10.1016/0022-510x(84)90033-9.
The growth "in vitro" of muscle cells from 12 patients with Duchenne muscular dystrophy (DMD) was compared with that of muscle cells from 20 age-matched controls. In the DMD explants, the lag phase (3 days) was shorter than in controls (6 days). In dissociated cells, plating efficiency (20%) and doubling time (30 h) were identical in DMD and controls. In cultures from three DMD patients, cell clusters were occasionally observed. Myotube morphometry showed significant abnormalities in DMD cultures: the number of myotubes per field was 8.2 +/- 0.8 and 26.7 +/- 0.6 in controls, P less than 0.001; myotube length (151 +/- 20 micron) and diameter (8.2 +/- 0.9 micron) in DMD cultures were half the control values (312 +/- 46 micron and 15.6 +/- 1.2 micron, respectively, P less than 0.001). The number of nuclei per myotube in DMD was one-quarter of that in control muscle (4.0 +/- 0.2 vs 15.8 +/- 2.2, P less than 0.001). It is concluded that DMD cultures show cellular heterogeneity with the presence of fibroblasts and non-fusing myoblasts; furthermore they show delayed myoblast fusion and poor myotube differentiation.
对12例杜氏肌营养不良症（DMD）患者的肌肉细胞“体外”生长情况与20例年龄匹配的对照者的肌肉细胞生长情况进行了比较。在DMD外植体中，延迟期（3天）比对照组（6天）短。在解离细胞中，DMD组和对照组的接种效率（20%）和倍增时间（30小时）相同。在3例DMD患者的培养物中，偶尔观察到细胞簇。肌管形态测量显示DMD培养物存在显著异常：对照组每视野肌管数量为26.7±0.6个，DMD组为8.2±0.8个，P<0.001；DMD培养物中肌管长度（151±20微米）和直径（8.2±0.9微米）分别为对照值的一半（分别为312±46微米和15.6±1.2微米），P<0.001。DMD中每个肌管的核数量为对照肌肉的四分之一（4.0±0.2对15.8±2.2，P<0.001）。结论是，DMD培养物显示出细胞异质性，存在成纤维细胞和不融合的成肌细胞；此外，它们还显示出成肌细胞融合延迟和肌管分化不良。
