Article
CuII(atsm) improves the neurological phenotype and survival of SOD1G93A mice and selectively increases enzymatically active SOD1 in the spinal cord.
Scientific reports - 13 Feb 2017
Hilton James B, Mercer Stephen W, Lim Nastasia K H, Faux Noel G, Buncic Gojko, Beckman Joseph S, Roberts Blaine R, Donnelly Paul S, White Anthony R, Crouch Peter J
Abstract excerpt
Ubiquitous expression of mutant Cu/Zn-superoxide dismutase (SOD1) selectively affects motor neurons in the central nervous system (CNS), causing the adult-onset degenerative disease amyotrophic lateral sclerosis (ALS). The CNS-specific impact of ubiquitous mutant SOD1 expression is recapitulated in transgenic mouse models of the disease. Here we present outcomes for the metallo-complex CuII(atsm) tested for...
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