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

Blaine R. Roberts,1* Nastasia K. H. Lim,2* Erin J. McAllum,2* Paul S. Donnelly,3,4 Dominic J. Hare,1,6 Philip A. Doble,6 Bradley J. Turner,1 Katherine A. Price,2 Sin Chun Lim,3,4 Brett M. Paterson,3,4 James L. Hickey,3,4 Timothy W. Rhoads,7,8 Jared R. Williams,7,8 Katja M. Kanninen,2 Lin W. Hung,1,4 Jeffrey R. Liddell,2 Alexandra Grubman,2 Jean-Francois Monty,10 Roxana M. Llanos,10 David R. Kramer,11 Julian F. B. Mercer,10 Ashley I. Bush,1 X Colin L. Masters,1 James A. Duce,1,12 Qiao-Xin Li,1 Joseph S. Beckman,7,8,9 Kevin J. Barnham,1,4,5 Anthony R. White,1,2 and Peter J. Crouch1,2 1Florey Institute of Neuroscience and Mental Health, 2Department of Pathology, 3School of Chemistry, 4Bio21 Institute of Biotechnology and Molecular Biology, and 5Department of Pharmacology, The University of Melbourne, Parkville, 3010 Victoria, Australia, 6Elemental Bio-imaging Facility, and Department of Chemistry and Forensic Science, University of Technology Sydney, 2007 Broadway, New South Wales, Australia, 7Department of Biochemistry and Biophysics, 8Linus Pauling Institute, and 9Environmental Health Sciences Centre, Oregon State University, Corvallis, Oregon 97331, 10Centre for Cellular and Molecular Biology, School of Life and Environmental Sciences, Deakin University, Burwood, 3125 Victoria, Australia, 11ANU Medical School, ANU College of Medicine, Biology and the Environment, The Australian National University, 0200 Canberra, Australian Capital Territory, Australia, and 12School of Molecular and Cellular Biology, University of Leeds, Leeds LS2 9JT, United Kingdom