Journal article

Endogenous Cu in the central nervous system fails to satiate the elevated requirement for Cu in a mutant SOD1 mouse model of ALS

JB Hilton, AR White, PJ Crouch

METALLOMICS | ROYAL SOC CHEMISTRY | Published : 2016

Abstract

Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease, a fatal degenerative disorder in which motor neurons in the central nervous system (CNS) progressively deteriorate. Most cases of ALS are sporadic, but 10% are familial and mutations affecting the copper (Cu)-dependent antioxidant Cu/Zn-superoxide dismutase (SOD1) are the most common familial cause. Cu malfunction is evident in CNS tissue from transgenic mice that over-express mutant SOD1 and modulating Cu bioavailability in the CNS provides positive therapeutic outcomes. In the present study we assessed levels of Cu and Zn, SOD activity, and SOD1 protein levels in CNS and non-CNS tissue from transgenic muta..

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Grants

Awarded by Australian National Health and Medical Research Council (NHMRC)


Awarded by NHMRC R.D. Wright Biomedical Research Fellowship (CDF2)


Funding Acknowledgements

All ICP-MS analyses were performed at the Biometals Facility, Florey Institute of Neurosciences and Mental Health by Ms Irene Volitakis. The research was supported by funds from the Australian National Health and Medical Research Council (NHMRC Grant 1061550) and the University of Melbourne. JBH is recipient of the Australian Postgraduate Award and the Nancy Frances Curry Scholarship. PJC is recipient of the NHMRC R.D. Wright Biomedical Research Fellowship (CDF2, 1084927).