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Themed collection Mammalian Copper Transport and Related Disorders

22 items
Editorial

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.

Graphical abstract: Mammalian copper biology: hitting the pause button in celebration of three pioneers and four decades of discovery
Editorial

Reflections of a cupromaniac

Joseph Prohaska reflects on a career in copper research.

Graphical abstract: Reflections of a cupromaniac
Editorial

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.

Graphical abstract: Copper comes of age in Melbourne
Perspective

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.

Graphical abstract: The copper rush of the nineties
Minireview

Copper trafficking to the secretory pathway

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

Graphical abstract: Copper trafficking to the secretory pathway
From the themed collection: Metallomics Recent HOT articles
Minireview

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.

Graphical abstract: Non-ceruloplasmin bound copper and ATP7B gene variants in Alzheimer's disease
Minireview

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.

Graphical abstract: Metallo-pathways to Alzheimer's disease: lessons from genetic disorders of copper trafficking
Minireview

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.

Graphical abstract: The emerging role of lysosomes in copper homeostasis
From the themed collection: Metallomics Recent HOT articles
Critical Review

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.

Graphical abstract: Bacterial Cu+-ATPases: models for molecular structure–function studies
Critical Review

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.

Graphical abstract: Ceruloplasmin and other copper binding components of blood plasma and their functions: an update
From the themed collection: Metallomics Recent HOT articles
Critical Review

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.

Graphical abstract: Copper and conquer: copper complexes of di-2-pyridylketone thiosemicarbazones as novel anti-cancer therapeutics
Communication

A ratiometric fluorescent sensor for the mitochondrial copper pool

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

Graphical abstract: A ratiometric fluorescent sensor for the mitochondrial copper pool
Communication

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.

Graphical abstract: A systems biology approach reveals new endoplasmic reticulum-associated targets for the correction of the ATP7B mutant causing Wilson disease
From the themed collection: Metallomics Recent HOT articles
Paper

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.

Graphical abstract: Knockdown of copper-transporting ATPase 1 (Atp7a) impairs iron flux in fully-differentiated rat (IEC-6) and human (Caco-2) intestinal epithelial cells
Paper

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.

Graphical abstract: Copper, zinc and calcium: imaging and quantification in anterior pituitary secretory granules
Paper

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.

Graphical abstract: Ceruloplasmin is regulated by copper and lactational hormones in PMC42-LA mammary epithelial cell culture models
Open Access Paper

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.

Graphical abstract: Endogenous Cu in the central nervous system fails to satiate the elevated requirement for Cu in a mutant SOD1 mouse model of ALS
Open Access Paper

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.

Graphical abstract: Multiple di-leucines in the ATP7A copper transporter are required for retrograde trafficking to the trans-Golgi network
Paper

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.

Graphical abstract: Characterizing the molecular phenotype of an Atp7aT985I conditional knock in mouse model for X-linked distal hereditary motor neuropathy (dHMNX)
Paper

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.

Graphical abstract: Copper transporters and chaperones CTR1, CTR2, ATOX1, and CCS as determinants of cisplatin sensitivity
Paper

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.

Graphical abstract: Decreased copper concentrations but increased lysyl oxidase activity in ischemic hearts of rhesus monkeys
Paper

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.

Graphical abstract: Copper-responsive gene expression in the methanotroph Methylosinus trichosporium OB3b
22 items

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.

Spotlight

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