Anatomical redistribution of endogenous copper in embryonic mice overexpressing SOD1

K Kysenius; JB Hilton; B Paul; DJ Hare; PJ Crouch

Journal article

Anatomical redistribution of endogenous copper in embryonic mice overexpressing SOD1

K Kysenius, JB Hilton, B Paul, DJ Hare, PJ Crouch

METALLOMICS | ROYAL SOC CHEMISTRY | Published : 2019

DOI: 10.1039/c8mt00242h

Abstract

Mutations in the copper (Cu)- and zinc (Zn)-binding metalloenzyme Cu/Zn-superoxide dismutase (SOD1) cause familial forms of amyotrophic lateral sclerosis (ALS), a fatal adult-onset neurodegenerative disorder of the central nervous system (CNS). Transgenic over-expression of mutant SOD1 produces a robust ALS-like phenotype in mice. Despite being ubiquitously expressed from the moment of conception, the mechanisms underlying the CNS-selective phenotype of mutant SOD1 expression remain poorly understood. We have previously shown that the physiological requirement for copper in SOD1 is unsatiated in the CNS of adult mice overexpressing mutant SOD1 and that suboptimal delivery of Cu to SOD1 in th..

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University of Melbourne Researchers

Bence Paul Author Geography, Earth and Atmospheric Sciences

James Hilton Author Biochemistry and Pharmacology

Peter Crouch Author Biochemistry and Pharmacology

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Grants

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

Awarded by NHMRC

Funding Acknowledgements

This research was supported by funds from an Australian National Health and Medical Research Council (NHMRC; Project Grant GNT1061550 - PJC), the University of Melbourne, the Motor Neurone Disease Research Institute of Australia (Betty Laidlaw MND Research Grant DJH, PJC; Jenny Simko Research Grant - DJH, PJC), and Parkinson's Victoria (Argyrou Family Fellowship - DJH). K. K. was recipient of the Sigrid Juselius Postdoctoral Fellowship. J. B. H. was recipient of the Australian Postgraduate Award and the Nancy Frances Curry Scholarship. P. J. C. is recipient of the NHMRC R. D. Wright Biomedical Research Fellowship (CDF2, 1084927). DJH is supported by an NHMRC Career Development Fellowship - Industry (GNT1122981) in partnership with Agilent Technologies. This study utilised the Australian Phenomics Network Histopathology and Organ Pathology Service, University of Melbourne.