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首页> 外文期刊>Biochemical Genetics >Expression of Human A4V Mutant Cu,Zn Superoxide Dismutase in Schizosaccharomyces pombe: Investigations of its Toxic Properties
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Expression of Human A4V Mutant Cu,Zn Superoxide Dismutase in Schizosaccharomyces pombe: Investigations of its Toxic Properties

机译：人A4V突变型铜锌超氧化物歧化酶在粟酒裂殖酵母中的表达：其毒性研究

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摘要

Cu,Zn superoxide dismutase (SOD1) is an antioxidant enzyme that catalyzes the removal of superoxide radicals generated in various biological oxidations. Amyotrophic lateral sclerosis (ALS) is one of the most common neurodegenerative disorders, occurring in families (FALS) and sporadically (SALS). FALS and SALS are distinguishable genetically but not clinically. More than 100 point mutations in the human SOD 1 gene have been identified that cause FALS. In order to determine the effects of mutant SOD protein, we first cloned wild-type and A4V mutant human SOD1 into Schizosaccharomyces pombe. This study shows viabilities and some antioxidant properties including SOD, catalase, proteasomal activity, and protein carbonyl levels of transformants in SOD1 deleted strain (MN415); and its parental strain (JY741) at different stress conditions. There was no more oxidative damage in the human mutant SOD carrying the transformant strain compared with other strains. These results may help to explain whether ALS progresses as a consequence of cellular oxidative damage. Keywords Copper–zinc superoxide dismutase - A4V mutant human SOD1 - Amyotrophic lateral sclerosis - Fission yeast - Schizosaccharomyces pombe
机译：铜锌超氧化物歧化酶（SOD1）是一种抗氧化酶，可催化去除各种生物氧化反应中产生的超氧化物自由基。肌萎缩性侧索硬化症（ALS）是最常见的神经退行性疾病之一，发生于家庭（FALS）和偶发性（SALS）。 FALS和SALS在遗传上可区分，但在临床上不可区分。已经确定了人类SOD 1基因中的100多个点突变会导致FALS。为了确定突变型SOD蛋白的作用，我们首先将野生型和A4V突变型人SOD1克隆到粟酒裂殖酵母中。这项研究显示了缺失的SOD1菌株（MN415）的活力和一些抗氧化特性，包括SOD，过氧化氢酶，蛋白酶体活性和转化子的蛋白质羰基水平；以及在不同胁迫条件下的亲本菌株（JY741）。与其他菌株相比，携带转化株的人突变型SOD中没有更多的氧化损伤。这些结果可能有助于解释ALS是否由于细胞氧化损伤而进展。关键词铜锌超氧化物歧化酶-A4V突变型人SOD1-肌萎缩性侧索硬化-裂变酵母-裂殖酵母

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