James Hilton

Superoxide Dismutase 1 in Health and Disease: How a Frontline Antioxidant Becomes Neurotoxic

Benjamin G Trist, James B Hilton, Dominic J Hare, Peter J Crouch, Kay L Double

Journal article  |  2020  |  Angewandte Chemie International Edition

Cu/Zn superoxide dismutase (SOD1) is a frontline antioxidant enzyme catalysing superoxide breakdown and is important for most form..

TDP-43 Triggers Mitochondrial DNA Release via mPTP to Activate cGAS/STING in ALS

Chien-Hsiung Yu, Sophia Davidson, Cassandra R Harapas, James B Hilton, Michael J Mlodzianoski, Pawat Laohamonthonkul, Cynthia Louis, Ronnie Ren Jie Low, Jonas Moecking, Dominic De Nardo, Katherine R Balka, Dale J Calleja, Fiona Moghaddas, Erya Ni, Catriona A McLean, Andre L Samson, Shiraz Tyebji, Christopher J Tonkin, Christopher R Bye, Bradley J Turner, et al.

Journal article  |  2020  |  Cell

Cytoplasmic accumulation of TDP-43 is a disease hallmark for many cases of amyotrophic lateral sclerosis (ALS), associated with a ..

A versatile quantitative microdroplet elemental imaging method optimised for integration in biochemical workflows for low-volume samples

Kai Kysenius, Bence Paul, James B Hilton, Jeffrey R Liddell, Dominic J Hare, Peter J Crouch

Journal article  |  2019  |  Analytical and Bioanalytical Chemistry

Anatomical redistribution of endogenous copper in embryonic mice overexpressing SOD1

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

Journal article  |  2019  |  Metallomics

The accumulation of enzymatically inactive cuproenzymes is a CNS-specific phenomenon of the SOD1(G37R) mouse model of ALS and can be restored by overexpressing the human copper transporter hCTR1

James B Hilton, Kai Kysenius, Anthony R White, Peter J Crouch

Journal article  |  2018  |  Experimental Neurology

Cu-II(atsm) improves the neurological phenotype and survival of SOD1(G93A) mice and selectively increases enzymatically active SOD1 in the spinal cord

James B Hilton, Stephen W Mercer, Nastasia KH Lim, Noel G Faux, Gojko Buncic, Joseph S Beckman, Blaine R Roberts, Paul S Donnelly, Anthony R White, Peter J Crouch

Journal article  |  2017  |  SCIENTIFIC REPORTS

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

Journal article  |  2016  |  Metallomics

Cuproenzyme dysfunction in the pathogenesis of amyotrophic lateral sclerosis and multiple sclerosis

James Benjamin William Hilton

Thesis / Dissertation  |  2016

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterised by the selective death of motor neurons wit..

Metal-deficient SOD1 in amyotrophic lateral sclerosis

James B Hilton, Anthony R White, Peter J Crouch

Journal article  |  2015  |  Journal of Molecular Medicine

Localized changes to glycogen synthase kinase-3 and collapsin response mediator protein-2 in the Huntington's disease affected brain

Nastasia KH Lim, Lin W Hung, Terence Y Pang, Catriona A Mclean, Jeffrey R Liddell, James B Hilton, Qiao-Xin Li, Anthony R White, Anthony J Hannan, Peter J Crouch

Journal article  |  2014  |  Human Molecular Genetics