The role of copper homeostasis in brain disease

Y An, S Li, X Huang, X Chen, H Shan… - International journal of …, 2022 - mdpi.com
In the human body, copper is an important trace element and is a cofactor for several
important enzymes involved in energy production, iron metabolism, neuropeptide activation …

The endolysosomal system and proteostasis: from development to degeneration

B Winckler, V Faundez, S Maday, Q Cai… - Journal of …, 2018 - Soc Neuroscience
How do neurons adapt their endolysosomal system to address the particular challenge of
membrane transport across their elaborate cellular landscape and to maintain proteostasis …

Elesclomol alleviates Menkes pathology and mortality by escorting Cu to cuproenzymes in mice

LM Guthrie, S Soma, S Yuan, A Silva, M Zulkifli… - Science, 2020 - science.org
Loss-of-function mutations in the copper (Cu) transporter ATP7A cause Menkes disease.
Menkes is an infantile, fatal, hereditary copper-deficiency disorder that is characterized by …

Copper regulates rest-activity cycles through the locus coeruleus-norepinephrine system

T **ao, CM Ackerman, EC Carroll, S Jia… - Nature chemical …, 2018 - nature.com
The unusually high demand for metals in the brain, along with insufficient understanding of
how their dysregulation contributes to neurological diseases, motivates the study of how …

Copper dyshomoeostasis in Parkinson's disease: implications for pathogenesis and indications for novel therapeutics

KM Davies, JFB Mercer, N Chen, KL Double - Clinical Science, 2016 - portlandpress.com
Copper is a biometal essential for normal brain development and function, thus copper
deficiency or excess results in central nervous system disease. Well-characterized disorders …

ATP7A-regulated enzyme metalation and trafficking in the menkes disease puzzle

N Horn, P Wittung-Stafshede - Biomedicines, 2021 - mdpi.com
Copper is vital for numerous cellular functions affecting all tissues and organ systems in the
body. The copper pump, ATP7A is critical for whole-body, cellular, and subcellular copper …

Chelating principles in Menkes and Wilson diseases: Choosing the right compounds in the right combinations at the right time

N Horn, LB Møller, VM Nurchi, J Aaseth - Journal of inorganic biochemistry, 2019 - Elsevier
Dysregulation of copper homeostasis in humans is primarily found in two genetic diseases
of copper transport, Menkes and Wilson diseases, which show symptoms of copper …

The elements of life: A biocentric tour of the periodic table

KA Remick, JD Helmann - Advances in microbial physiology, 2023 - Elsevier
Living systems are built from a small subset of the atomic elements, including the bulk
macronutrients (C, H, N, O, P, S) and ions (Mg, K, Na, Ca) together with a small but variable …

Ratiometric two-photon microscopy reveals attomolar copper buffering in normal and Menkes mutant cells

MT Morgan, D Bourassa… - Proceedings of the …, 2019 - National Acad Sciences
Copper is controlled by a sophisticated network of transport and storage proteins within
mammalian cells, yet its uptake and efflux occur with rapid kinetics. Present as Cu (I) within …

Is copper a new target to counteract the progression of chronic diseases?

C Balsano, C Porcu, S Sideri - Metallomics, 2018 - academic.oup.com
Altered regulation of copper (Cu) homeostasis may contribute to the development of many
pathologies, such as metabolic, cardiovascular, neurodegenerative and cancerous …