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Oral Treatment with CuII(atsm) Increases Mutant SOD1 In Vivo but Protects Motor Neurons and Improves the Phenotype of a Transgenic Mouse Model of Amyotrophic Lateral Sclerosis

Roberts, Blaine R.; Lim, Nastasia K. H.; McAllum, Erin J.; Donnelly, Paul S; Hare, Dominic J.; Doble, Philip A.; Turner, Bradley; Price, Katherine A.; Lim, Sin Chun; Paterson, Brett M.; Kramer, David


Mutations in the metallo-protein Cu/Zn-superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS) in humans and an expression level-dependent phenotype in transgenic rodents. We show that oral treatment with the therapeutic agent diacetyl-bis(4-methylthiosemicarbazonato)copperII [CuII(atsm)] increased the concentration of mutant SOD1 (SOD1G37R) in ALS model mice, but paradoxically improved locomotor function and survival of the mice. To determine why the mice with increased levels of...[Show more]

CollectionsANU Research Publications
Date published: 2014
Type: Journal article
Source: Journal of Neuroscience
DOI: 10.1523/JNEUROSCI.4196-13.2014


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