Copper homeostasis as a therapeutic target in amyotrophic lateral sclerosis with SOD1 mutations

Eiichi Tokuda, Yoshiaki Furukawa

研究成果: Article査読

抄録

Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disease affecting both upper and lower motor neurons, and currently, there is no cure or effective treatment. Mutations in a gene encoding a ubiquitous antioxidant enzyme, Cu,Zn-superoxide dismutase (SOD1), have been first identified as a cause of familial forms of ALS. It is widely accepted that mutant SOD1 proteins cause the disease through a gain in toxicity but not through a loss of its physiological function. SOD1 is a major copper-binding protein and regulates copper homeostasis in the cell; therefore, a toxicity of mutant SOD1 could arise from the disruption of copper homeostasis. In this review, we will briefly review recent studies implying roles of copper homeostasis in the pathogenesis of SOD1-ALS and highlight the therapeutic interventions focusing on pharmacological as well as genetic regulations of copper homeostasis to modify the pathological process in SOD1-ALS.

本文言語English
論文番号636
ジャーナルInternational Journal of Molecular Sciences
17
5
DOI
出版ステータスPublished - 2016 5月 1

ASJC Scopus subject areas

  • 物理化学および理論化学
  • 有機化学
  • 分光学
  • 無機化学
  • 触媒
  • 分子生物学
  • コンピュータ サイエンスの応用

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