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Insights into the mechanisms of copper dyshomeostasis in amyotrophic lateral sclerosis

  • Francisco J Gil-Bea (a1) (a2) (a3), Garazi Aldanondo (a1) (a3) (a4), Haizpea Lasa-Fernández (a1) (a3) (a5), Adolfo López de Munain (a1) (a3) (a5) (a6) and Ainara Vallejo-Illarramendi (a1) (a3)...
Abstract

Amyotrophic lateral sclerosis (ALS) is a severe neuromuscular disease characterised by a progressive loss of motor neurons that usually results in paralysis and death within 2 to 5 years after disease onset. The pathophysiological mechanisms involved in ALS remain largely unknown and to date there is no effective treatment for this disease. Here, we review clinical and experimental evidence suggesting that dysregulation of copper homeostasis in the central nervous system is a crucial underlying event in motor neuron degeneration and ALS pathophysiology. We also review and discuss novel approaches seeking to target copper delivery to treat ALS. These novel approaches may be clinically relevant not only for ALS but also for other neurological disorders with abnormal copper homeostasis, such as Parkinson's, Huntington's and Prion diseases.

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Corresponding author
*Corresponding authors: F. Gil-Bea and A. Vallejo, Instituto BioDonostia, Hospital Universitario Donostia, San Sebastián, P° Doctor Begiristain s/n, 20014 San Sebastián, Spain. Tel: +34 943006128; Fax: +34 943006250; E-mail: ainaravallejo@yahoo.es, francisco.gilbea@biodonostia.org
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Authors contributed equally to this work.

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References
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