An attempt to treat amyotrophic lateral sclerosis by intracellular copper modification using ammonium tetrathiomolybdate and/or metallothionein: Fundamentals and perspective

Shin Ichi Ono, Ei Ichi Tokuda, Eriko Okawa, Shunsuke Watanabe

研究成果: Chapter

1 被引用数 (Scopus)

抄録

Mutation in superoxide diamutasel (SOD1) is a cause of hereditary form of amyotrophic lateral sclerosis (ALS). Novel acquired toxicity (gain-of-function) is believed to play a crucial role. We propose that the nature of mutant SOD1 toxicity is disruption of intracellular Cu homeostasis. We provide evidences that copper transporters and chaperons are geared to accumulate Cu ion in the cells, and its excretion is downregulated with mutant SOD1 ("intracellular copper dysregulation" theory). Intracellular Cu modification using a Cu chelator and/or metallothionein resulted in a favorable outcome in an experimental study with a rodent model for hereditary form of ALS.

本文言語English
ホスト出版物のタイトルBrain Diseases and Metalloproteins
出版社Pan Stanford Publishing Pte. Ltd.
ページ367-405
ページ数39
ISBN(印刷版)9789814316019
DOI
出版ステータスPublished - 2012 7月 31
外部発表はい

ASJC Scopus subject areas

  • 生化学、遺伝学、分子生物学(全般)
  • 医学(全般)

フィンガープリント

「An attempt to treat amyotrophic lateral sclerosis by intracellular copper modification using ammonium tetrathiomolybdate and/or metallothionein: Fundamentals and perspective」の研究トピックを掘り下げます。これらがまとまってユニークなフィンガープリントを構成します。

引用スタイル