Immunochemical characterization on pathological oligomers of mutant Cu/Zn-superoxide dismutase in amyotrophic lateral sclerosis
作者: Eiichi TokudaItsuki AnzaiTakao NomuraKeisuke ToichiMasahiko WatanabeShinji OharaSeiji WatanabeKoji YamanakaYuta MorisakiHidemi MisawaYoshiaki Furukawa
作者单位: 1Keio University
2Hokkaido University Graduate School of Medicine
3Matsumoto Medical Center
4Research Institute of Environmental Medicine, Nagoya University
刊名: Molecular Neurodegeneration, 2017, Vol.12 (1)
来源数据库: Springer Journal
DOI: 10.1186/s13024-016-0145-9
关键词: Amyotrophic lateral sclerosisCu/Zn-superoxide dismutaseProtein misfoldingDisulfide bond
英文摘要: Dominant mutations in Cu/Zn-superoxide dismutase ( SOD1 ) gene cause a familial form of amyotrophic lateral sclerosis ( SOD1 -ALS) with accumulation of misfolded SOD1 proteins as intracellular inclusions in spinal motor neurons. Oligomerization of SOD1 via abnormal disulfide crosslinks has been proposed as one of the misfolding pathways occurring in mutant SOD1; however, the pathological relevance of such oligomerization in the SOD1 -ALS cases still remains obscure.
原始语种摘要: Dominant mutations in Cu/Zn-superoxide dismutase ( SOD1 ) gene cause a familial form of amyotrophic lateral sclerosis ( SOD1 -ALS) with accumulation of misfolded SOD1 proteins as intracellular inclusions in spinal motor neurons. Oligomerization of SOD1 via abnormal disulfide crosslinks has been proposed as one of the misfolding pathways occurring in mutant SOD1; however, the pathological relevance of such oligomerization in the SOD1 -ALS cases still remains obscure.
全文获取路径: Springer  (合作)
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来源刊物:
影响因子:4.007 (2012)

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关键词翻译
关键词翻译
  • superoxide 过氧化物
  • sclerosis 硬化
  • dismutase 歧化酶
  • amyotrophic 肌肉萎缩的
  • pathological 病理学的
  • lateral 侧面的
  • SOD Superintendent Of Documents
  • mutant 突变体
  • ALS Accumulator Left Shift
  • intracellular 胞内的