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多胺修饰的过氧化氢酶作为过氧化氢和一氧化氮清除剂在家族性肌萎缩侧索硬化转基因小鼠中的治疗益处。

Therapeutic benefit of polyamine-modified catalase as a scavenger of hydrogen peroxide and nitric oxide in familial amyotrophic lateral sclerosis transgenics.

作者信息

Poduslo J F, Whelan S L, Curran G L, Wengenack T M

机构信息

Department of Neurology, Mayo Clinic and Foundation, Rochester, MN 55905, USA.

出版信息

Ann Neurol. 2000 Dec;48(6):943-7.

Abstract

Continuous subcutaneous administration of polyamine-modified catalase that has increased permeability at the blood-brain barrier showed both a highly significant delay in onset and an increase in survival in a transgenic mouse model of familial amyotrophic lateral sclerosis having a point mutation in the gene encoding copper/zinc superoxide dismutase. These results suggest that hydrogen peroxide-mediated oxidative stress with subsequent free radical damage involving nitric oxide and possibly hydroxyl radicals in motor neurons may be the culprit in familial amyotrophic lateral sclerosis.

摘要

在编码铜/锌超氧化物歧化酶的基因中存在点突变的家族性肌萎缩侧索硬化转基因小鼠模型中,持续皮下注射在血脑屏障处通透性增加的多胺修饰过氧化氢酶,结果显示发病显著延迟且生存期延长。这些结果表明,过氧化氢介导的氧化应激以及随后运动神经元中涉及一氧化氮和可能的羟基自由基的自由基损伤,可能是家族性肌萎缩侧索硬化的病因。

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