Article
Oral treatment with Cu(II)(atsm) increases mutant SOD1 in vivo but protects motor neurons and improves the phenotype of a transgenic mouse model of amyotrophic lateral sclerosis.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 4 Jun 2014
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, Crouch Peter J
Abstract excerpt
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(II) [Cu(II)(atsm)] increased the concentration of mutant SOD1 (SOD1G37R) in ALS model mice, but paradoxically improved...
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