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杜兴氏肌营养不良犬类同源物中的肌纤维损伤与再生

Myofiber injury and regeneration in a canine homologue of Duchenne muscular dystrophy.

作者信息

Childers M K, Okamura C S, Bogan D J, Bogan J R, Sullivan M J, Kornegay J N

机构信息

Department of Physical Medicine and Rehabilitation, School of Medicine, University of Missouri-Columbia, 65212,USA.

出版信息

Am J Phys Med Rehabil. 2001 Mar;80(3):175-81. doi: 10.1097/00002060-200103000-00004.

Abstract

OBJECTIVE

To test the hypothesis that differential skeletal muscle involvement, previously observed in dogs with a homologue of Duchenne muscular dystrophy, correlates with the histochemical markers of myofiber injury and regeneration.

DESIGN

Evidence of injury (cellular penetration by Evans blue dye, immunoglobulin G expression, hematoxylin and eosin staining of necrotic figures), myofiber regeneration (fetal myosin heavy chain isoform expression), and morphologic indices in the cranial sartorius (CS), long digital extensor, and vastus lateralis muscles were examined in five dogs with dystrophy and five normal dogs.

RESULTS

Only the CS muscle, at 1 mo, demonstrated significant differences in injury when compared with age-matched controls. By 6 mo, the long digital extensor and vastus lateralis also suffered greater than normal injury. Only the dystrophic CS tissue expressed a notable increase in mean myofiber diameter when compared with other muscles at 6 mo. Normal CS muscles revealed a distinct population of small myofibers at this age.

CONCLUSION

The CS seems unique in its selective pathologic involvement. These differences may contribute to the marked regenerative response of this muscle in the dystrophic state. An improved understanding of mechanisms by which some dystrophin-deficient canine muscles remain spared from injury may provide clues to investigate and prevent the degenerative processes in humans.

摘要

目的

验证以下假说,即在患有杜兴氏肌营养不良症同源疾病的犬类中先前观察到的骨骼肌差异受累情况,与肌纤维损伤和再生的组织化学标志物相关。

设计

在五只患有肌营养不良症的犬和五只正常犬中,检查了损伤证据(伊文思蓝染料的细胞穿透、免疫球蛋白G表达、坏死形态的苏木精和伊红染色)、肌纤维再生(胎儿肌球蛋白重链同工型表达)以及颅侧缝匠肌(CS)、趾长伸肌和股外侧肌的形态学指标。

结果

仅在1个月时,CS肌肉与年龄匹配的对照组相比,在损伤方面表现出显著差异。到6个月时,趾长伸肌和股外侧肌也遭受了比正常情况更严重的损伤。与6个月时的其他肌肉相比,仅营养不良的CS组织的平均肌纤维直径有显著增加。正常的CS肌肉在这个年龄显示出一群独特的小肌纤维。

结论

CS在其选择性病理受累方面似乎具有独特性。这些差异可能导致该肌肉在营养不良状态下出现明显的再生反应。更好地理解一些肌营养不良蛋白缺乏的犬类肌肉未受损伤的机制,可能为研究和预防人类的退化过程提供线索。

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