TDP-43 triggers mitochondrial DNA release via mPTP to activate cGAS/STING in ALS CH Yu, S Davidson, CR Harapas, JB Hilton, MJ Mlodzianoski, ... Cell 183 (3), 636-649. e18, 2020 | 653 | 2020 |
Superoxide dismutase 1 in health and disease: how a frontline antioxidant becomes neurotoxic BG Trist, JB Hilton, DJ Hare, PJ Crouch, KL Double Angewandte Chemie International Edition 60 (17), 9215-9246, 2021 | 136 | 2021 |
CuII(atsm) improves the neurological phenotype and survival of SOD1G93A mice and selectively increases enzymatically active SOD1 in the spinal cord JB Hilton, SW Mercer, NKH Lim, NG Faux, G Buncic, JS Beckman, ... Scientific reports 7 (1), 42292, 2017 | 84 | 2017 |
Metal-deficient SOD1 in amyotrophic lateral sclerosis JB Hilton, AR White, PJ Crouch Journal of Molecular Medicine 93, 481-487, 2015 | 72 | 2015 |
Localized changes to glycogen synthase kinase-3 and collapsin response mediator protein-2 in the Huntington's disease affected brain NKH Lim, LW Hung, TY Pang, CA Mclean, JR Liddell, JB Hilton, QX Li, ... Human Molecular Genetics 23 (15), 4051-4063, 2014 | 50 | 2014 |
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 8 (9), 1002-1011, 2016 | 33 | 2016 |
Copper-ATSM as a treatment for ALS: support from mutant SOD1 models and beyond S Nikseresht, JBW Hilton, K Kysenius, JR Liddell, PJ Crouch Life 10 (11), 271, 2020 | 28 | 2020 |
A versatile quantitative microdroplet elemental imaging method optimised for integration in biochemical workflows for low-volume samples K Kysenius, B Paul, JB Hilton, JR Liddell, DJ Hare, PJ Crouch Analytical and bioanalytical chemistry 411, 603-616, 2019 | 23 | 2019 |
The accumulation of enzymatically inactive cuproenzymes is a CNS-specific phenomenon of the SOD1G37R mouse model of ALS and can be restored by overexpressing the human copper … JB Hilton, K Kysenius, AR White, PJ Crouch Experimental Neurology 307, 118-128, 2018 | 22 | 2018 |
Disrupted copper availability in sporadic ALS: Implications for CuII(atsm) as a treatment option JBW Hilton, K Kysenius, JR Liddell, C Rautengarten, SW Mercer, B Paul, ... BioRxiv, 2020.04. 17.047704, 2020 | 12 | 2020 |
An integrated mass spectrometry imaging and digital pathology workflow for objective detection of colorectal tumours by unique atomic signatures B Paul, K Kysenius, JB Hilton, MWM Jones, RW Hutchinson, ... Chemical Science 12 (30), 10321-10333, 2021 | 11 | 2021 |
Evidence for disrupted copper availability in human spinal cord supports CuII(atsm) as a treatment option for sporadic cases of ALS JBW Hilton, K Kysenius, JR Liddell, SW Mercer, B Paul, JS Beckman, ... Scientific Reports 14 (1), 5929, 2024 | 9 | 2024 |
Proteome-wide systems genetics identifies UFMylation as a regulator of skeletal muscle function J Molendijk, R Blazev, RJ Mills, YK Ng, KI Watt, D Chau, P Gregorevic, ... Elife 11, e82951, 2022 | 8 | 2022 |
Anatomical redistribution of endogenous copper in embryonic mice overexpressing SOD1 K Kysenius, JB Hilton, B Paul, DJ Hare, PJ Crouch Metallomics 11 (1), 141-150, 2019 | 8 | 2019 |
Microglial ferroptotic stress causes non-cell autonomous neuronal death JR Liddell, JBW Hilton, K Kysenius, JL Billings, S Nikseresht, LE McInnes, ... Molecular Neurodegeneration 19 (1), 14, 2024 | 7 | 2024 |
CuII(atsm) significantly decreases microglial reactivity in patients with sporadic amyotrophic lateral sclerosis. JR Liddell, JBW Hilton, PJ Crouch Neuropathology & Applied Neurobiology 49 (5), 2023 | 6 | 2023 |
Evidence for decreased copper associated with demyelination in the corpus callosum of cuprizone-treated mice JBW Hilton, K Kysenius, JR Liddell, SW Mercer, DJ Hare, G Buncic, ... Metallomics 16 (1), mfad072, 2024 | 5 | 2024 |
Fundamental Neurochemistry Review: Copper availability as a potential therapeutic target in progressive supranuclear palsy: Insight from other neurodegenerative diseases JL Billings, JBW Hilton, JR Liddell, DJ Hare, PJ Crouch Journal of Neurochemistry 167 (3), 337-346, 2023 | 3 | 2023 |
Transdermal application of soluble CuII (atsm) increases brain and spinal cord uptake compared to gavage with an insoluble suspension S Nikseresht, JBW Hilton, JR Liddell, K Kysenius, AI Bush, S Ayton, ... Neuroscience 509, 125-131, 2023 | 3 | 2023 |
Sex-dependent effects of amyloid precursor-like protein 2 in the SOD1-G37R transgenic mouse model of MND PH Truong, PJ Crouch, JBW Hilton, CA McLean, R Cappai, GD Ciccotosto Cellular and Molecular Life Sciences 78 (19-20), 6605-6630, 2021 | 2 | 2021 |