A gain-of-function of an amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in K_m for hydrogen peroxide

Moon Bin Yim; Jung-Hoon Kang; Hyung-Soon Yim

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A gain-of-function of an amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in K_m for hydrogen peroxide

机译：肌萎缩性侧索硬化相关的铜，锌超氧化物歧化酶突变体的功能增强：由于过氧化氢的K_m降低，自由基形成的增强

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Cu,Zn-superoxide dismutase (SOD) is known to be a locus of mutation in familial amyotrophic lateral sclerosis (FALS). Transgenic mice that express a mutant Cu,Zn-SOD, Gly-93 - Ala (G93A), have been shown to develop amyotrophic lateral sclerosis (ALS) symptoms. We cloned the FALS mutant, G93A, and wild-type cDNA of human Cu,Zn-SOD, overexpressed them in Sf9 insect cells, purified the proteins, and studied their enzymic activities for cata- lyzing the dismutation of superoxide anions and the genera- tion of free radicals with H_2O_2 as substrate.

机译：铜锌超氧化物歧化酶（SOD）是家族性肌萎缩性侧索硬化症（FALS）的突变位点。表达突变型Cu，Zn-SOD，Gly-93-Ala（G93A）的转基因小鼠已显示出肌萎缩性侧索硬化症（ALS）症状。我们克隆了人Cu，Zn-SOD的FALS突变体G93A和野生型cDNA，使其在Sf9昆虫细胞中过表达，纯化了这些蛋白，并研究了它们的酶活性以催化超氧阴离子的歧化和H_2O_2为底物的自由基的合成

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《Proceedings of the National Academy of Sciences of the United States of America》 |1996年第12期|p.5709-5714|共6页
作者
Moon Bin Yim; Jung-Hoon Kang; Hyung-Soon Yim;
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收录信息美国《科学引文索引》(SCI);美国《生物学医学文摘》(MEDLINE);美国《化学文摘》(CA);
原文格式 PDF
正文语种 eng
中图分类自然科学总论;
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专利

1. Release of Copper Ions form the Familial Amyotrophic Lateral Sclerosis-associated Cu, Zn-Superoxide Dismutase Mutants [J] . Won Sik Eum, Jung Hoon Kang Molecules and cells . 1999,第1期

机译：铜离子从家族性肌萎缩性侧索硬化症相关的铜，锌超氧化物歧化酶突变体的释放。
2. The free radical-generating function of a familial amyotrophic lateralsclerosis-associated D90A Cu,Zn-superoxide dismutase mutant [J] . Kim SM., Eum WS., Kwon OB., Biochemistry and Molecular Biology International . 1998,第6期

机译：家族性肌萎缩性侧索硬化症相关的D90A Cu，Zn超氧化物歧化酶突变体的自由基产生功能
3. Heterodimer formation of wild-type and amyotrophic lateral sclerosis-causing mutant Cu/Zn-superoxide dismutase induces toxicity independent of protein aggregation. [J] . Witan H, Kern A, Koziollek-Drechsler I, Human Molecular Genetics . 2008,第10期

机译：导致野生型和肌萎缩性侧索硬化的异二聚体形成的突变型Cu / Zn-超氧化物歧化酶诱导了独立于蛋白质聚集的毒性。
4. Identification of Three Mutations in the Cu,Zn-Superoxide Dismutase (Cu,Zn-SOD) Gene with Familial Amyotrophic Lateral Sclerosis: Transduction of Human Cu,Zn-SOD into PC12 Cells by HIV-1 TAT Protein Basic Domain [C] . CHIH-MING CHOU, CHANG-JEN HUANG, CHWEN-MING SHIH, Asian Society for Mitochondrial Research and Medicine . 2005

机译：鉴定Cu，Zn-超氧化物歧化酶（Cu，Zn-SOD）基因的三种突变与家族性肌肌疏远硬化剂：HIV-1 TAT蛋白碱性结构域的转导，Zn-SOD进入PC12细胞
5. Thermodynamics, kinetics and structural dynamics of amyotrophic lateral sclerosis-associated mutant copper-zinc superoxide dismutases. [D] . Rumfeldt, Jessica Anne Osborne. 2006

机译：肌萎缩性侧索硬化相关突变铜锌超氧化物歧化酶的热力学，动力学和结构动力学。
6. A gain-of-function of an amyotrophic lateral sclerosis-associated CuZn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in Km for hydrogen peroxide. [O] . M B Yim, J H Kang, H S Yim, 1996

机译：肌萎缩性侧索硬化相关的铜锌超氧化物歧化酶突变体的功能增强：由于过氧化氢的Km降低自由基形成的增强。
7. A gain-of-function of an amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in Km for hydrogen peroxide. [O] . Yim, M B, Kang, J H, Yim, H S, 1996

机译：肌萎缩性侧索硬化相关的铜，锌超氧化物歧化酶突变体的功能增强：由于过氧化氢的Km降低，自由基形成的增强。

A gain-of-function of an amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in K_m for hydrogen peroxide

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