SMALL-CALIBER SKELETAL-MUSCLE FIBERS DO NOT SUFFER DELETERIOUS CONSEQUENCES OF DYSTROPHIC GENE-EXPRESSION
SMALL-CALIBER SKELETAL-MUSCLE FIBERS DO NOT SUFFER DELETERIOUS CONSEQUENCES OF DYSTROPHIC GENE-EXPRESSION
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DOI：
10.1002/ajmg.1320250407
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发表时间：
1986-12-01
期刊：
影响因子：
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通讯作者：
CARPENTER, S
中科院分区：
文献类型：
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作者：
KARPATI, G;CARPENTER, S
在Duchenne营养不良以及CHF-147仓鼠和MDX小鼠的遗传性营养不良中，营养不良基因表达的基本有害后果是骨骼肌纤维节段性坏死。基因缺陷的性质和肌肉纤维损伤的发病机制尚不清楚。然而，临床和实验证据表明，周长低于一定水平(营养不良仓鼠和mdx鼠的直径约为20-25μm)的肌纤维不容易坏死。这种明显的“免疫力”已经在自然较小周长的肌肉纤维(如眼外肌)中观察到，并在通过实验程序(仓鼠和小鼠)或病理过程(杜兴患者)阻止正常生长的纤维中观察到。小口径肌肉纤维抵抗营养不良基因表达的坏死性影响的细胞或分子基础尚不清楚。在小口径肌肉纤维中，单位表面积的正常收缩相关机械应变相对较小；这可以解释一些营养不良患者对坏死的相对抵抗力。
In Duchenne dystrophy and in the genetic dystrophies of CHF‐147 hamsters and MDX mice, the fundamental deleterious consequence of dystrophic gene expression issegmental necrosis of skeletal muscle fibers. The nature of the gene defects and the pathogenesis of muscle fiber damage are not known. However, clinical and experimental evidence indicates that muscle fibers whose girth is below a certain level (estimated at approximately 20–25 μm in diameter in dystrophic hamsters and MDX mice) arenotsusceptible to necrosis. This apparent “immunity” has been observed in muscle fibers that are naturally of small girth (such as those in extraocular muscles), and in fibers that were prevented from growing normally by experimental procedures (hamsters and mice) or by pathological processes (Duchenne patients). The cellular or molecular basis by which small‐caliber muscle fibers are resistant to the necrotizing effect of the dystrophic gene expression remains unknown. In small‐caliber muscle fibers, the normal contraction‐related mechanical strains per unit surface area are relatively less than in larger fibers; this could explain their relative resistance to necrosis in some dystrophies.