Themed collection Mammalian Copper Transport and Related Disorders

Guest editors Sharon La Fontaine, Richard Burke and David Giedroc introduce this themed collection of Metallomics on Mammalian Copper Transport and Related Disorders.

Joseph Prohaska reflects on a career in copper research.

Julian Mercer and Jim Camakaris discuss the history and impact at the international level of copper research in Australia and particularly Melbourne.

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 (Cu) is indispensible for growth and development of human organisms.

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.

Copper is an essential metal ion that provides catalytic function to numerous enzymes and also regulates protein trafficking, neurotransmission and intracellular signaling.

The lysosomal system operates as a focal point where a number of important physiological processes such as endocytosis, autophagy and nutrient sensing converge.

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.

We know that blood plasma contains many proteins and also other components that bind copper.

Copper is an essential trace metal required by organisms to perform a number of important biological processes.

Ratiometric probe for Cu(I) reveals influence of cisplatin on mitochondrial copper homeostasis.

This study provides new insights into understanding the interplay between ER quality control and copper transport mechanisms in Wilson disease.

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.

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.

PMC42-LA three-dimensional organoids reminiscent of mammary gland alveoli secrete copper-bound ceruloplasmin (sCP) in both lactating and suckled models.

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.

The ATP7A protein is a ubiquitous copper-transporting P-type ATPase that is mutated in the lethal pediatric disorder of copper metabolism, Menkes disease.

We have generated an Atp7a conditional knock in mouse model of dHMNX expressing Atp7a^T985I, the orthologue of the human ATP7A^T994I identified in dHMNX patients.

We used CRISPR-Cas9 technology to address whether copper transporters or chaperones CTR1, CTR2, ATOX1, and CCS mediate cisplatin sensitivity in human cells.

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.

Gene expression and bioinformatics studies provide new insight into copper homeostasis in the methanotroph Methylosinus trichosporium OB3b.