ZnII(atsm) is protective in amyotrophic lateral sclerosis model mice via a copper delivery mechanism

EJ McAllum; BR Roberts; JL Hickey; TN Dang; A Grubman; PS Donnelly; JR Liddell; AR White; PJ Crouch

Journal article

ZnII(atsm) is protective in amyotrophic lateral sclerosis model mice via a copper delivery mechanism

EJ McAllum, BR Roberts, JL Hickey, TN Dang, A Grubman, PS Donnelly, JR Liddell, AR White, PJ Crouch

Neurobiology of Disease | ACADEMIC PRESS INC ELSEVIER SCIENCE | Published : 2015

DOI: 10.1016/j.nbd.2015.02.023

Abstract

Mutations in the metalloprotein Cu,Zn-superoxide dismutase (SOD1) cause approximately 20% of familial cases of amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease for which effective therapeutics do not yet exist. Transgenic rodent models based on over-expression of mutant SOD1 have been developed and these have provided opportunity to test new therapeutic strategies and to study the mechanisms of mutant SOD1 toxicity. Although the mechanisms of mutant SOD1 toxicity are yet to be fully elucidated, incorrect or incomplete metallation of SOD1 confers abnormal folding, aggregation and biochemical properties, and improving the metallation state of SOD1 provides a viable therap..

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University of Melbourne Researchers

Paul Donnelly Author

Jeffrey Liddell Author

Peter Crouch Author

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Grants

Awarded by National Science Foundation

Funding Acknowledgements

Data for the vehicle treated mice presented in this study have been published previously (McAllum et al., 2013) and have been reproduced with permission from the publisher Informa Healthcare. Data for the Zn<SUP>II</SUP>(atsm) treated mice presented in this study were collected from a single study that involved the vehicle treated and Cu<SUP>II</SUP>(atsm) treated mice as described (McAllum et al., 2013). The research was supported by funds from the National Health and Medical Research Council (1005651 and 1061550), the Australian Research Council (DP110101368), and the University of Melbourne.