Special Issue "Metals in Neurodegenerative Diseases"

A special issue of Inorganics (ISSN 2304-6740). This special issue belongs to the section "Bioinorganic Chemistry".

Deadline for manuscript submissions: 30 June 2019

Special Issue Editor

Guest Editor
Prof. Dr. Blaine Roberts

The Florey Institute of Neuroscience and Mental Health, The University of Melbourne, Parkville, Victoria, Australia
Website | E-Mail
Interests: metalloproteomics; mass spectrometry; Neurodegeneration; Alzheimer’s disease; amyotrophic lateral sclerosis; amyloid beta; biomarkers; native protein purification

Special Issue Information

Dear Colleagues,

The human brain is perhaps the most complex organ in existence. In addition to this, it has considerable and varied metabolic requirements that must be consistently maintained throughout the lifespan of an individual. This is underlined by the fact that it consumes approximately 25% of an individual’s daily energy resources, despite accounting for only 2% of total body weight. As a direct consequence of high nutrient input, the brain is rich in essential elements (particularly Fe, Cu, and Zn), with concentrations in some regions of the brain equalling or exceeding those found in the liver. Alarmingly, during neurodegeneration, the balance of essential trace elements and the metalloenzymes that use them is disrupted. Although the measurement of total essential element abundances is important, it only yields a fraction of the story. Currently, our understanding of the relationships between changes in trace elements and the function of their related metalloproteins is limited. In this Special Issue, we highlight the most current discoveries in this area: (1) The consequences of metal mis-incorporation and absence to proper protein structure and function, (2) The recent advances made in speciation techniques and their application to direct measurement of metalloenzymes, and (3) New therapeutic strategies aimed at targeting metal dyshomeostasis.

Prof. Dr. Blaine Roberts
Guest Editor

Manuscript Submission Information

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  • metalloproteins/metalloenzymes
  • neurodegeneration
  • essential minerals
  • trace elements
  • metal protein attenuating compounds
  • copper
  • iron
  • zinc
  • Alzheimer’s Disease
  • Parkinson’s Disease
  • amyotrophic lateral sclerosis
  • Wilson’s disease
  • Menkes disease

Published Papers (1 paper)

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Open AccessReview
Laser Ablation Inductively Coupled Plasma Spectrometry: Metal Imaging in Experimental and Clinical Wilson Disease
Received: 12 March 2019 / Revised: 14 April 2019 / Accepted: 17 April 2019 / Published: 19 April 2019
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Wilson disease is an inherited disorder caused by mutations in the ATP7B gene resulting in copper metabolism disturbances. As a consequence, copper accumulates in different organs with most common presentation in liver and brain. Chelating agents that nonspecifically chelate copper, and promote its [...] Read more.
Wilson disease is an inherited disorder caused by mutations in the ATP7B gene resulting in copper metabolism disturbances. As a consequence, copper accumulates in different organs with most common presentation in liver and brain. Chelating agents that nonspecifically chelate copper, and promote its urinary excretion, or zinc salts interfering with the absorption of copper from the gastrointestinal tract, are current medications. Also gene therapy, restoring ATP7B gene function or trials with bis-choline tetrathiomolybdate (WTX101) removing excess copper from intracellular hepatic copper stores and increasing biliary copper excretion, is promising in reducing body’s copper content. Therapy efficacy is mostly evaluated by testing for evidence of liver disease and neurological symptoms, hepatic synthetic functions, indices of copper metabolisms, urinary copper excretions, or direct copper measurements. However, several studies conducted in patients or Wilson disease models have shown that not only the absolute concentration of copper, but also its spatial distribution within the diseased tissue is relevant for disease severity and outcome. Here we discuss laser ablation inductively coupled plasma spectrometry imaging as a novel method for accurate determination of trace element concentrations with high diagnostic sensitivity, spatial resolution, specificity, and quantification ability in experimental and clinical Wilson disease specimens. Full article
(This article belongs to the Special Issue Metals in Neurodegenerative Diseases)

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