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肌萎缩性侧索硬化症相关的Cu,Zn-SOD 被引量：3

Cu,Zn-superoxide Dismutase Associated with Amyotrophic Lateral Sclerosis
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摘要 超氧化物歧化酶(superoxide dismutase,SOD)被称为生物体内自由基的清洁剂,其主要形式Cu,Zn-SOD称SOD1.SOD1突变体可引起致死性运动神经元疾病肌萎缩性侧索硬化症(ALS).但是,SOD1的毒性机理尚未完全清楚.本文概述了SOD1、Cu分子伴侣(copper chaperonefor SOD1,CCS)的分子结构和CCS活化SOD1的机理,重点分析了突变体SOD1构象变化的原因及其在ALS中的可能致病机制的最新研究进展. Superoxide dismutases （SOD） arc known as biological cleaning agents of free radicals in vivo,and Cu, Zn-SOD also called SOD1, is one of the main forms. SOD1 mutations have been linked fatal human motor neuron diseases, such as amyotrophic lateral sclerosis, ALS. However, the precise roles of SOD1 in ALS occurenace and progression remianed unknown. This review will focus on the mechanism of SODI＇ s activation based on the structural conformation analysis of SOD1 and the copper chaperone for SOD1 （CCS）. Meanwhile, the latest research progresses of the possible pathogenic mechanisms in ALS will also be discussed.
作者 丘创华 周勇军 侯敢 黄迪南
机构地区 广东医学院生物化学与分子生物学教研室
出处 《中国生物化学与分子生物学报》 CAS CSCD 北大核心 2008年第2期107-112,共6页 Chinese Journal of Biochemistry and Molecular Biology
基金 广东省自然科学基金(No.04011397 No.5011588)~~
关键词 超氧化物歧化酶1(SOD1) 突变 聚集 肌萎缩性侧索硬化症 Cu, Zn-superoxide dismutase （ SOD1 ） mutation aggregation amyotrophic lateral sclerosis （ALS）
分类号 Q74 [生物学—分子生物学]
  • 相关文献

参考文献33

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  • 2林庆斌,廖升荣,熊亚红,乐学义.超氧化物歧化酶(SOD)的研究和应用进展[J].化学世界,2006,47(6):378-381. 被引量：77
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二级参考文献20

共引文献76

同被引文献38

  • 1黄慧,张成.家族性ALS的SOD1基因突变及发病机制研究进展[J].国外医学（神经病学．神经外科学分册）,2004,31(6):511-514. 被引量：5
  • 2Siun CT,Beow CY.DNA RNA and Protein Extraction:The Past and The Present[J].Journal of Biomedicine and Biotechnology,2009,11:1-10.
  • 3Vlug AS,Jaarsma D.Long term proteasome inhibition does not preferentially afflict motor neurons in organotypical spinal cordeultures[J].Amyotroph Lateral Scler Other Motor Neuron Disord,2004,5(1):16-21.
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  • 5Carroll MC, Gironard JB, Ulloa JL. Mechanisms for activating Cu-and Zn-containing superoxide dismutase in the absence of the CCS Cu chaperone. Proc Natl Acad Sci USA,2004, 101:5964- 5969.
  • 6Valentine JS, Doucette PA, Zittin PS. Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis. Anan Rev Biochem, 2005, 74:563.
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  • 8Furukawa Y, O'Halloran T. Posttranslational modifications in Cu, Zn-superoxide dismutase and mutations associated with amyotmphic lateral sclerosis. Antioxid Redox Signal,2006, 8 (5- 6 ) : 847-867.
  • 9Bruijn LI, Houseweart MK, Kato S, et al. Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1. Science, 1998, 281:1851-1854.
  • 10Reaume AG, Elliott JL, Hoffman EK, et al. Motor neurons in Cu/Zn superoxide dismutase-deficient mice develops normally but exhibit enhanced cell death after axonal injury. Nat Genet, 1996, 13:43-47.

引证文献3

二级引证文献7

中国生物化学与分子生物学报
2008年 第2期

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