Insights into the mechanisms of copper dyshomeostasis in amyotrophic lateral sclerosis

• Published in: Expert reviews in molecular medicine Vol. 19; p. e7
• Main Authors: Gil-Bea, Francisco J, Aldanondo, Garazi, Lasa-Fernández, Haizpea, López de Munain, Adolfo, Vallejo-Illarramendi, Ainara
• Format: Journal Article
• Language: English
• Published: Cambridge, UK Cambridge University Press 09.06.2017
• Subjects: Amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - etiology; Amyotrophic Lateral Sclerosis - metabolism; Animals; Biological Transport; Blood-brain barrier; Brain; Central nervous system; Central Nervous System - metabolism; Colleges & universities; Copper; Copper - metabolism; Cytochrome; Death; Degeneration; Disease; Enzymes; Exports; Free radicals; Homeostasis; Humans; Medicin och hälsovetenskap; Mitochondria - genetics; Mitochondria - metabolism; Motor neurons; Motor Neurons - metabolism; Motor task performance; Movement disorders; Nervous system; Nervous System Diseases - etiology; Nervous System Diseases - metabolism; Neurobiology; Neurochemistry; Neurodegenerative diseases; Neurological diseases; Neurology; Neurons; Neurosciences; Oxidation-Reduction; Paralysis; Parkinson's disease; Proteins; Review; Reviews; Rodents; Sclerosis
• ISSN: 1462-3994; 1462-3994
• DOI: 10.1017/erm.2017.9

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.