DUCHENNE MUSCULAR-DYSTROPHY - PLASMA-MEMBRANE LOSS INITIATES MUSCLE-CELL NECROSIS UNLESS IT IS REPAIRED
DUCHENNE MUSCULAR-DYSTROPHY - PLASMA-MEMBRANE LOSS INITIATES MUSCLE-CELL NECROSIS UNLESS IT IS REPAIRED
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DOI：
10.1093/brain/102.1.147
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发表时间：
1979-01-01
期刊：
影响因子：
14.5
通讯作者：
KARPATI, G
中科院分区：
文献类型：
--
作者：
CARPENTER, S;KARPATI, G
对30名患有杜氏肌营养不良症的男孩的活检研究表明，肌细胞坏死是由质膜的丧失开始的，随后，在短时间间隔后，Z盘溶解和线粒体改变构成了完全发展的坏死。含有胶原蛋白的空基板管表明再生可能无法发生。这些管是胶原沉积的基础。有证据表明，小片的膜损失可以修复，虽然表面的细胞质丢失了一片，基底膜脱落了一片。明显过度收缩的纤维未表现出坏死的特征。
Study of biopsies from 30 boys with Duchenne muscular dystrophy suggests that necrosis of muscle cells is initiated by loss of plasma membrane, followed, after a short interval, by Z disc lysis and mitochondrial changes to constitute the picture of fully developed necrosis. Empty basal lamina tubes containing collagen indicated that regeneration may fail to occur. The tubes form a basis for collagen deposition. Evidence suggests that small patches of membrane loss can be repaired, though a slice of superficial cytoplasm is lost, and a piece of detached basal lamina results. The markedly hypercontracted fibres seen did not show features of necrosis.