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REVIEW article

Front. Med.

Sec. Hepatobiliary Diseases

Volume 12 - 2025 | doi: 10.3389/fmed.2025.1673283

This article is part of the Research TopicNeurological Involvement in Heavy Metal Accumulation and Neurotoxin ExposureView all 6 articles

The Role of Copper Dysregulation in Wilson Disease: An Expert Opinion

Provisionally accepted
  • 1Private Practice for Internal Medicine, Baden-Baden, Germany
  • 2Institute of Clinical Chemistry and Pathobiochemistry, RWTH Aachen University, Aachen, NRW, Germany

The final, formatted version of the article will be published soon.

The handling of free copper is a crucial aspect of copper metabolism. Any dysfunction in this process can lead to the pathophysiology of diseases, such as Wilson disease. This disorder, characterized by an excess of copper in the liver, occurs when the body is unable to excrete copper into bile. The symptoms of the disease result from the accumulation of free copper in liver cells, leading to hepatocellular injury and the release of copper into the bloodstream. This, in turn, causes damage in other areas of the body, such as the brain. The primary goal of therapy is to convert toxic free copper into harmless complexes, rather than simply removing copper from the body.

Keywords: Liver, Brain, ATP7B, Mutation, Copper, Wilson disease

Received: 28 Jul 2025; Accepted: 06 Oct 2025.

Copyright: © 2025 STREMMEL and Weiskirchen. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Ralf Weiskirchen, rweiskirchen@ukaachen.de

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