title: Recent progress towards an effective treatment of amyotrophic lateral sclerosis using the SOD1 mouse model in a preclinical setting.
abstract: Amyotrophic lateral sclerosis (ALS) is a progressive, fatal and incurable neurodegenerative disorder. Motor neurone degeneration can be caused by genetic mutation but the exact etiology of the disease, particularly for sporadic illness, still remains unclear. Therapeutics which target known pathogenic mechanisms involved in ALS, such as protein aggregation, oxidative stress, apoptosis, inflammation, endoplasmic reticulum stress and mitochondria dysfunction, are currently being pursued in order to provide neuroprotection which may be able to slow down, or perhaps even halt, disease progression. This present review focuses on the compounds which have been recently evaluated using the SOD1 mouse model, the most widely used preclinical model for ALS research.
authors.affiliation: Department of Chemistry and Physics, La Trobe Institute for Molecular Science, La Trobe University, Melbourne, Victoria 3086, Australia.
authors.forename: Elisse C
authors.initials: EC
authors.lastname: Browne
authors.affiliation: Department of Chemistry and Physics, La Trobe Institute for Molecular Science, La Trobe University, Melbourne, Victoria 3086, Australia. Electronic address: b.abbott@latrobe.edu.au.
authors.forename: Belinda M
authors.initials: BM
authors.lastname: Abbott
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000188:drug therapy* / Q000235:genetics; D000818:Animals; D001688:Biological Products / Q000627:therapeutic use; D004195:Disease Models, Animal; D055808:Drug Discovery; D006801:Humans; D051379:Mice; D000072105:Superoxide Dismutase-1 / Q000235:genetics*
chemical_list: D001688:Biological Products; D000072105:Superoxide Dismutase-1
keywords: Amyotrophic lateral sclerosis; Drug treatment; Motor neurone disease; SOD1 mouse model