Tracing copper-thiomolybdate complexes in a prospective treatment for Wilson's disease

Limei Zhang, Josef Lichtmannegger, Karl H. Summer, Samuel Webb, Ingrid J. Pickering, Graham N. George

Research output: Contribution to journalArticle

52 Citations (Scopus)

Abstract

Wilson's disease is a human genetic disorder which results in copper accumulation in liver and brain. Treatments such as copper chelation therapy or dietary supplementation with zinc can ameliorate the effects of the disease, but if left untreated, it results in hepatitis, neurological complications, and death. Tetrathiomolybdate (TTM) is a promising new treatment for Wilson's disease which has been demonstrated both in an animal model and in clinical trials. X-ray absorption spectroscopy suggests that TTM acts as a novel copper chelator, forming a complex with accumulated copper in liver. We have used X-ray absorption spectroscopy and X-ray fluorescence imaging to trace the molecular form and distribution of the complex in liver and kidney of an animal model of human Wilson's disease. Our work allows new insights into metabolism of the metal complex in the diseased state.

Original languageEnglish (US)
Pages (from-to)891-897
Number of pages7
JournalBiochemistry
Volume48
Issue number5
DOIs
StatePublished - Feb 10 2009

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Hepatolenticular Degeneration
Copper
X-Ray Absorption Spectroscopy
Liver
X ray absorption spectroscopy
Animals
Animal Models
Chelation Therapy
Inborn Genetic Diseases
Optical Imaging
Coordination Complexes
Medical Genetics
Therapeutics
Chelating Agents
Dietary Supplements
Chelation
Metabolism
Hepatitis
Zinc
Brain

ASJC Scopus subject areas

  • Biochemistry

Cite this

Zhang, L., Lichtmannegger, J., Summer, K. H., Webb, S., Pickering, I. J., & George, G. N. (2009). Tracing copper-thiomolybdate complexes in a prospective treatment for Wilson's disease. Biochemistry, 48(5), 891-897. https://doi.org/10.1021/bi801926e

Tracing copper-thiomolybdate complexes in a prospective treatment for Wilson's disease. / Zhang, Limei; Lichtmannegger, Josef; Summer, Karl H.; Webb, Samuel; Pickering, Ingrid J.; George, Graham N.

In: Biochemistry, Vol. 48, No. 5, 10.02.2009, p. 891-897.

Research output: Contribution to journalArticle

Zhang, L, Lichtmannegger, J, Summer, KH, Webb, S, Pickering, IJ & George, GN 2009, 'Tracing copper-thiomolybdate complexes in a prospective treatment for Wilson's disease', Biochemistry, vol. 48, no. 5, pp. 891-897. https://doi.org/10.1021/bi801926e
Zhang L, Lichtmannegger J, Summer KH, Webb S, Pickering IJ, George GN. Tracing copper-thiomolybdate complexes in a prospective treatment for Wilson's disease. Biochemistry. 2009 Feb 10;48(5):891-897. https://doi.org/10.1021/bi801926e
Zhang, Limei ; Lichtmannegger, Josef ; Summer, Karl H. ; Webb, Samuel ; Pickering, Ingrid J. ; George, Graham N. / Tracing copper-thiomolybdate complexes in a prospective treatment for Wilson's disease. In: Biochemistry. 2009 ; Vol. 48, No. 5. pp. 891-897.
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