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杜兴氏肌营养不良症的病理损伤可能是由于缺乏抗肌萎缩蛋白导致细胞内氧自由基生成增加,以及随后钙代谢的改变所致。

The pathological damage in Duchenne muscular dystrophy may be due to increased intracellular OXY-radical generation caused by the absence of dystrophin and subsequent alterations in Ca2+ metabolism.

作者信息

Baker M S, Austin L

机构信息

Department of Biochemistry, Monash University, Clayton, Australia.

出版信息

Med Hypotheses. 1989 Jul;29(3):187-93. doi: 10.1016/0306-9877(89)90193-x.

Abstract

Recent advances in the genetic and molecular pathogenesis of Duchenne muscular dystrophy and the evidence suggesting a role for oxygen free radicals (oxy-radicals) in the development of this disease are reviewed. In addition, we outline a working of hypothesis as to how disruptions in intracellular Ca2+ homeostasis within the dystrophic cell may initiate cycles of increased oxy-radical fluxes within these cells, leading to intracellular oxidative damage.

摘要

本文综述了杜氏肌营养不良症遗传和分子发病机制的最新进展,以及提示氧自由基在该疾病发展中起作用的证据。此外,我们概述了一个关于营养不良细胞内细胞内钙离子稳态破坏如何引发这些细胞内氧自由基通量增加的循环,从而导致细胞内氧化损伤的工作假说。

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