Copper homeostasis and cuproptosis in health and disease

L Chen, J Min, F Wang - Signal transduction and targeted therapy, 2022 - nature.com
As an essential micronutrient, copper is required for a wide range of physiological processes
in virtually all cell types. Because the accumulation of intracellular copper can induce …

Molecular mechanisms of TDP-43 misfolding and pathology in amyotrophic lateral sclerosis

A Prasad, V Bharathi, V Sivalingam… - Frontiers in molecular …, 2019 - frontiersin.org
TAR DNA binding protein 43 (TDP-43) is a versatile RNA/DNA binding protein involved in
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …

The molecular mechanisms of copper metabolism and its roles in human diseases

J Chen, Y Jiang, H Shi, Y Peng, X Fan, C Li - Pflügers Archiv-European …, 2020 - Springer
Copper is an essential element in cells; it can act as either a recipient or a donor of
electrons, participating in various reactions. However, an excess of copper ions in cells is …

Ferroptosis mediates selective motor neuron death in amyotrophic lateral sclerosis

T Wang, D Tomas, ND Perera, B Cuic… - Cell Death & …, 2022 - nature.com
Amyotrophic lateral sclerosis (ALS) is caused by selective degeneration of motor neurons in
the brain and spinal cord; however, the primary cell death pathway (s) mediating motor …

The molecular and cellular basis of copper dysregulation and its relationship with human pathologies

MT Maung, A Carlson, M Olea‐Flores… - The FASEB …, 2021 - Wiley Online Library
Copper (Cu) is an essential micronutrient required for the activity of redox‐active enzymes
involved in critical metabolic reactions, signaling pathways, and biological functions …

Copper ions and Parkinson's disease: why is homeostasis so relevant?

M Bisaglia, L Bubacco - Biomolecules, 2020 - mdpi.com
The involvement of copper in numerous physiological processes makes this metal ion
essential for human life. Alterations in copper homeostasis might have deleterious …

Biomedical applications of copper ionophores

V Oliveri - Coordination Chemistry Reviews, 2020 - Elsevier
Copper dyshomeostasis is associated with a plethora of diseases, supporting the possibility
of applying copper ionophores to regenerate the metal homeostasis and treat copper …

CuII(atsm) inhibits ferroptosis: Implications for treatment of neurodegenerative disease

A Southon, K Szostak, KM Acevedo… - British journal of …, 2020 - Wiley Online Library
Background and Purpose Diacetyl‐bis (4‐methyl‐3‐thiosemicarbazonato) copperII (CuII
(atsm)) ameliorates neurodegeneration and delays disease progression in mouse models of …

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 …

Altered SOD1 maturation and post-translational modification in amyotrophic lateral sclerosis spinal cord

BG Trist, S Genoud, S Roudeau, A Rookyard… - Brain, 2022 - academic.oup.com
Aberrant self-assembly and toxicity of wild-type and mutant superoxide dismutase 1 (SOD1)
has been widely examined in silico, in vitro and in transgenic animal models of amyotrophic …