Superoxide dismutase 1 in health and disease: how a frontline antioxidant becomes neurotoxic

BG Trist, JB Hilton, DJ Hare, PJ Crouch… - Angewandte Chemie …, 2021 - Wiley Online Library
Cu/Zn superoxide dismutase (SOD1) is a frontline antioxidant enzyme catalysing superoxide
breakdown and is important for most forms of eukaryotic life. The evolution of aerobic …

Conservative iron chelation for neurodegenerative diseases such as Parkinson's disease and amyotrophic lateral sclerosis

D Devos, ZI Cabantchik, C Moreau, V Danel… - Journal of Neural …, 2020 - Springer
Focal iron accumulation associated with brain iron dyshomeostasis is a pathological
hallmark of various neurodegenerative diseases (NDD). The application of iron-sensitive …

Copper toxicity and deficiency: the vicious cycle at the core of protein aggregation in ALS

JH Min, H Sarlus, RA Harris - Frontiers in Molecular Neuroscience, 2024 - frontiersin.org
The pathophysiology of ALS involves many signs of a disruption in copper homeostasis, with
both excess free levels and functional deficiency likely occurring simultaneously. This is …

Copper metabolism and cuproptosis: molecular mechanisms and therapeutic perspectives in neurodegenerative diseases

X Ban, H Wan, X Wan, Y Tan, X Hu, H Ban… - Current medical …, 2024 - Springer
Copper is an essential trace element, and plays a vital role in numerous physiological
processes within the human body. During normal metabolism, the human body maintains …

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… - Scientific Reports, 2024 - nature.com
The copper compound CuII (atsm) has progressed to phase 2/3 testing for treatment of the
neurodegenerative disease amyotrophic lateral sclerosis (ALS). CuII (atsm) is …

Mass spectrometry imaging of SOD1 protein-metal complexes in SOD1G93A transgenic mice implicates demetalation with pathology

OJ Hale, TR Wells, RJ Mead, HJ Cooper - Nature Communications, 2024 - nature.com
Amyotrophic lateral sclerosis (ALS) is characterized by degeneration of motor neurons in the
central nervous system (CNS). Mutations in the metalloenzyme SOD1 are associated with …

Fundamental Neurochemistry Review: Copper availability as a potential therapeutic target in progressive supranuclear palsy: Insight from other neurodegenerative …

JL Billings, JBW Hilton, JR Liddell… - Journal of …, 2023 - Wiley Online Library
Since the first description of Parkinson's disease (PD) over two centuries ago, the
recognition of rare types of atypical parkinsonism has introduced a spectrum of related PD …

Emerging Roles of the Copper–CTR1 Axis in Tumorigenesis

Y Su, X Zhang, S Li, W Xie, J Guo - Molecular Cancer Research, 2022 - AACR
Physiologic roles of copper in metabolic homeostasis have been well established; however,
whether and how copper is dysregulated in tumors and contributes to tumorigenesis is not …

Copper-ATSM as a treatment for ALS: support from mutant SOD1 models and beyond

S Nikseresht, JBW Hilton, K Kysenius, JR Liddell… - Life, 2020 - mdpi.com
The blood–brain barrier permeant, copper-containing compound, CuII (atsm), has
successfully progressed from fundamental research outcomes in the laboratory through to …

Decreased spinal cord motor neuron numbers in mice depleted of central nervous system copper

JR Liddell, JBW Hilton, YJ Wang, JL Billings… - Metallomics, 2024 - academic.oup.com
Disrupted copper availability in the central nervous system (CNS) is implicated as a
significant feature of the neurodegenerative disease amyotrophic lateral sclerosis (ALS) …