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Int. J. Mol. Sci. 2016, 17(5), 636; doi:10.3390/ijms17050636

Copper Homeostasis as a Therapeutic Target in Amyotrophic Lateral Sclerosis with SOD1 Mutations

Laboratory for Mechanistic Chemistry of Biomolecules, Department of Chemistry, Keio University, Yokohama 223-8522, Japan
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Author to whom correspondence should be addressed.
Academic Editor: Masatoshi Maki
Received: 2 February 2016 / Revised: 3 April 2016 / Accepted: 22 April 2016 / Published: 28 April 2016
(This article belongs to the Special Issue Metalloproteins)
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Abstract

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. View Full-Text
Keywords: amyotrophic lateral sclerosis; Cu,Zn-superoxide dismutase; copper homeostasis amyotrophic lateral sclerosis; Cu,Zn-superoxide dismutase; copper homeostasis
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Tokuda, E.; Furukawa, Y. Copper Homeostasis as a Therapeutic Target in Amyotrophic Lateral Sclerosis with SOD1 Mutations. Int. J. Mol. Sci. 2016, 17, 636.

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