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Filtering by: Creator Grubman, Alexandra

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

Creator:

Roberts, Blaine R., Lim, Nastasia K. H., McAllum, Erin J., Donnelly, Paul S., Hare, Dominic J., Doble, Philip A., Turner, Bradley J., Price, Katherine A., Lim, Sin Chun, Paterson, Brett M., Hickey, James L., Rhoads, Timothy W., Williams, Jared R., Kanninen, Katja M., Hung, Lin W., Liddell, Jeffrey R., Grubman, Alexandra, Monty, Jean-Francois, Llanos, Roxana M., Kramer, David R., Mercer, Julian F. B., Bush, Ashley I., Masters, Colin L., Duce, James A., Li, Qiao-Xin, Beckman, Joseph S., Barnham, Kevin J., White, Anthony R., and Crouch, Peter J.

Abstract:

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)copper[superscript II] [Cu[superscript II](atsm)] increased the concentration of mutant SOD1 (SOD1G37R) in ALS model mice, but paradoxically improved locomotor...

Resource Type:

Article

Full Text:

,2 Lin W. Hung,1,4 Jeffrey R. Liddell,2 Alexandra Grubman,2 Jean-Francois Monty,10 Roxana M. Llanos