Mammalian copper biology: hitting the pause button in celebration of three pioneers and four decades of discovery
Guest editors Sharon La Fontaine, Richard Burke and David Giedroc introduce this themed collection of Metallomics on Mammalian Copper Transport and Related Disorders.
Reflections of a cupromaniac
Joseph Prohaska reflects on a career in copper research.
Copper comes of age in Melbourne
Julian Mercer and Jim Camakaris discuss the history and impact at the international level of copper research in Australia and particularly Melbourne.
The copper rush of the nineties
The discovery of copper-transporting ATPases in the 90s led to a molecular understanding of Menkes and Wilson disease. The historic events leading up to and following these discoveries are reviewed.
Copper trafficking to the secretory pathway
Copper (Cu) is indispensible for growth and development of human organisms.
Non-ceruloplasmin bound copper and ATP7B gene variants in Alzheimer's disease
ATP7B, a protein mainly expressed in the hepatocytes, is a copper chaperone that loads the metal into the serum copper–protein ceruloplasmin during its synthesis and also escorts superfluous copper into the bile, by a sophisticated trafficking mechanism.
Metallo-pathways to Alzheimer's disease: lessons from genetic disorders of copper trafficking
Copper is an essential metal ion that provides catalytic function to numerous enzymes and also regulates protein trafficking, neurotransmission and intracellular signaling.
The emerging role of lysosomes in copper homeostasis
The lysosomal system operates as a focal point where a number of important physiological processes such as endocytosis, autophagy and nutrient sensing converge.
Bacterial Cu+-ATPases: models for molecular structure–function studies
The characterization of bacterial Cu+-ATPases has significantly furthered our understanding of the structure, selectivity and transport mechanism of these enzymes, as well as their interplay with other elements of Cu+ distribution networks.
Ceruloplasmin and other copper binding components of blood plasma and their functions: an update
We know that blood plasma contains many proteins and also other components that bind copper.
Copper and conquer: copper complexes of di-2-pyridylketone thiosemicarbazones as novel anti-cancer therapeutics
Copper is an essential trace metal required by organisms to perform a number of important biological processes.
A ratiometric fluorescent sensor for the mitochondrial copper pool
Ratiometric probe for Cu(I) reveals influence of cisplatin on mitochondrial copper homeostasis.
A systems biology approach reveals new endoplasmic reticulum-associated targets for the correction of the ATP7B mutant causing Wilson disease
This study provides new insights into understanding the interplay between ER quality control and copper transport mechanisms in Wilson disease.
Knockdown of copper-transporting ATPase 1 (Atp7a) impairs iron flux in fully-differentiated rat (IEC-6) and human (Caco-2) intestinal epithelial cells
Silencing of the Atp7a copper-transporting ATPase impairs vectorial iron flux in fully-differentiated rat and human intestinal epithelial cells. These findings suggest that copper positively influences iron transport.
Copper, zinc and calcium: imaging and quantification in anterior pituitary secretory granules
Rodent pituitaries were subjected to subcellular fractionation. Secretory granule Cu, Zn and Ca levels were compared to levels of peptide amidating monooxygenase, a peptide processing enzyme requiring all three metals.
Ceruloplasmin is regulated by copper and lactational hormones in PMC42-LA mammary epithelial cell culture models
PMC42-LA three-dimensional organoids reminiscent of mammary gland alveoli secrete copper-bound ceruloplasmin (sCP) in both lactating and suckled models.
Endogenous Cu in the central nervous system fails to satiate the elevated requirement for Cu in a mutant SOD1 mouse model of ALS
It is unclear why ubiquitous expression of mutant SOD1 selectively affects the central nervous system in amyotrophic lateral sclerosis. Here we hypothesise that the central nervous system is primarily affected because, unlike other tissues, it has relatively limited capacity to satiate an increased requirement for Cu.
Multiple di-leucines in the ATP7A copper transporter are required for retrograde trafficking to the trans-Golgi network
The ATP7A protein is a ubiquitous copper-transporting P-type ATPase that is mutated in the lethal pediatric disorder of copper metabolism, Menkes disease.
Characterizing the molecular phenotype of an Atp7aT985I conditional knock in mouse model for X-linked distal hereditary motor neuropathy (dHMNX)
We have generated an Atp7a conditional knock in mouse model of dHMNX expressing Atp7aT985I, the orthologue of the human ATP7AT994I identified in dHMNX patients.
Copper transporters and chaperones CTR1, CTR2, ATOX1, and CCS as determinants of cisplatin sensitivity
We used CRISPR-Cas9 technology to address whether copper transporters or chaperones CTR1, CTR2, ATOX1, and CCS mediate cisplatin sensitivity in human cells.
Decreased copper concentrations but increased lysyl oxidase activity in ischemic hearts of rhesus monkeys
In response to ischemic conditions, Cu redistribution takes place although Cu concentration was decreased, leading to depressed Cu-dependent CCO activity but increased Cu-dependent LOX activity in the ischemic myocardium along with enhanced collagen deposition.
Copper-responsive gene expression in the methanotroph Methylosinus trichosporium OB3b
Gene expression and bioinformatics studies provide new insight into copper homeostasis in the methanotroph Methylosinus trichosporium OB3b.
About this collection
This themed issue, Guest Edited by Professors David Giedroc, Sharon La Fontaine and Richard Burke, marks the retirement of three pioneers in mammalian copper biology, Professors Jim Camakaris, Julian Mercer and Joseph Prohaska. This issue showcases research on the role of copper in health and disease, the chemistry and structural biology of key copper homeostasis proteins, the development of cutting edge imaging tools and therapies, and the model systems that have helped elucidate the function and regulation of this intriguing and exciting metal in the context of mammalian copper homeostasis.