INCREASED COLLAGEN-SYNTHESIS BY DUCHENNE MYOGENIC CLONES
INCREASED COLLAGEN-SYNTHESIS BY DUCHENNE MYOGENIC CLONES
复制标题
DOI：
10.1016/0022-510x(82)90220-9
复制
发表时间：
1982-01-01
影响因子：
4.4
通讯作者：
IONASESCU, R
中科院分区：
文献类型：
--
作者：
IONASESCU, V;IONASESCU, R
从3名Duchenne肌营养不良症患者、5名迟发性强直性肌营养不良症患者、2名脊髓性肌萎缩症患者、6名男性儿童对照、4名成人对照和6名正常流产胎儿（10-12周龄）中一式三份地生长肌源性细胞克隆。来自强直性肌营养不良和脊髓性肌萎缩患者的克隆具有正常的胶原合成。胎儿克隆显示3.3倍低于[3 H]细胞外（中）羟脯氨酸的值，而细胞内[3 H]羟脯氨酸水平显示正常值。[3 H]脯氨酸掺入的分析表明，[3 H]羟脯氨酸释放到杜氏肌细胞克隆的培养基中增加了4倍，而细胞内[3 H]羟脯氨酸水平显示正常值。Duchenne克隆的胶原更新正常。杜氏营养不良肌肉中胶原蛋白的增加量显然与疾病的原发性缺陷有关，而不是由于继发性替代纤维化。
Myogenic cell clones were grown in triplicates from 3 patients with Duchenne muscular dystrophy, 5 with late onset myotonic dystrophy, 2 with spinal muscular atrophy, 6 male children controls, 4 adult controls and 6 normal aborted fetuses (10-12 wk old). The clones from patients with myotonic dystrophy and spinal muscular atrophy had normal collagen synthesis. The fetal clones showed 3.3-fold lower values than control clones for [3H] extracellular (medium) hydroxyproline, while intracellular [3H]hydroxyproline levels showed normal values. Analysis of [3H]proline incorporation demonstrated a 4-fold increase in [3H]hydroxyproline released into the medium of Duchenne myogenic cell clones, while intracellular [3H]hydroxyproline levels showed normal values. The collagen turnover of Duchenne clones was normal. The increased amount of collagen present in Duchenne dystrophic muscle is evidently related to the primary defect of the disease and is not due to secondary replacement fibrosis.