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Potential of Curcumin As A Multifunctional Agent to Combat Wilson Disease

2010; Lippincott Williams & Wilkins; Volume: 51; Issue: 6 Linguagem: Inglês

10.1002/hep.23705

1527-3350

Peter V. E. van den Berghe, Roderick H.J. Houwen, Leo W. J. Klomp,

Drug Transport and Resistance Mechanisms

We thank Dr. Ji and Dr. Shen for mentioning other potential beneficial effects of curcumin on Wilson disease in addition to the improvement in the membrane expression of mutated copper-transporting adenosine triphosphatase (ATPase) ATP7B proteins that we described.1 Indeed, curcumin has drawn considerable attention recently as an agent with a broad range of properties that might be useful in liver diseases.2 The suggestion by Ji and Shen to conduct rigorous trials of curcumin in Wilson disease therefore seems timely. Nevertheless, some restraint seems appropriate. Even for decoppering agents that have proven effectiveness, such as D-penicillamine, zinc, and trientine, prospective, blinded, randomized clinical trials are lacking.3 As Wilson disease is rare, with an incidence of approximately 1:50,000, any prospective trial will face considerable organizational difficulties. Therefore, when trials are being developed, it seems proper to give priority to improving the evidence for established decoppering agents. We would therefore suggest that the potential of curcumin in Wilson disease should first be explored further in vitro. If it is proven effective, several animal models are available for both Wilson disease and other genetic diseases that cause copper overload (reviewed by Vonk et al.4), and they can subsequently be used to test the potential of curcumin to ameliorate or treat liver disease due to copper overload in vivo. Only when these results are clearly positive should human testing be considered. Dr. Peter V. E. van den Berghe M.D.* , Dr. Roderick H. J. Houwen M.D. , Dr. Leo W. J. Klomp M.D.* , * Departments of Metabolic and Endocrine Diseases, Metabolic Pediatric Gastroenterology, University Medical Center Utrecht, Utrecht, the Netherlands, Netherlands Metabolomics Center, Utrecht, the Netherlands.