Saito Y
J Neurol Sci. 1985 Apr;68(1):75-87. doi: 10.1016/0022-510x(85)90051-6.
Quantitative analyses of the muscle fibre type distribution and satellite cell population were performed on biceps muscles from control infants 6-30 months of age and patients with Werdnig-Hoffmann (WH) disease aged 1-13 months. In muscles of control infants, type 1 fibres ranged from 34.5 to 52.5% of a total of 500 muscle fibres, similar to the percentage in adult muscle. The distribution of type 2A and 2B fibres varied slightly at different ages (type 2A, 19.3-42.8%; type 2B, 21.1-39.2%). Type 2C fibres ranged from 0.2 to 3.4% and did not tend to decrease with age up to 30 months. The ratio of satellite cells to total subsarcolemmal nuclei was 8.4 +/- 1.6% (5-21 months). In muscles in Werdnig-Hoffmann (WH) disease, undifferentiated type 2C fibres were significantly increased in number, ranging from 10.4 to 84.2% (P less than 0.005). The prevalence of the fibres was not necessarily proportional to the clinical severity, the onset of the disease, or the age at biopsy. Satellite cells in WH disease were significantly increased in number (14.4 +/- 3.1%) in comparison with control infants (P less than 0.005). The satellite cells in WH disease appeared quiescent without morphological characteristics of "activation".
对6至30个月大的对照婴儿以及1至13个月大的韦尔尼克-霍夫曼（WH）病患者的二头肌进行了肌纤维类型分布和卫星细胞数量的定量分析。在对照婴儿的肌肉中，I型纤维占500条肌纤维总数的34.5%至52.5%，与成年肌肉中的百分比相似。2A型和2B型纤维的分布在不同年龄略有变化（2A型，19.3%至42.8%；2B型，21.1%至39.2%）。2C型纤维占0.2%至3.4%，在30个月之前并不随年龄增长而减少。卫星细胞与肌膜下总核的比例为8.4±1.6%（5至21个月）。在韦尔尼克-霍夫曼（WH）病患者的肌肉中，未分化的2C型纤维数量显著增加，占10.4%至84.2%（P<0.005）。这些纤维的患病率不一定与临床严重程度、疾病发作或活检时的年龄成比例。与对照婴儿相比，WH病患者的卫星细胞数量显著增加（14.4±3.1%）（P<0.005）。WH病患者的卫星细胞呈静止状态，没有“激活”的形态特征。
