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 journalArticlepeer-review

67 Scopus citations


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
Issue number5
StatePublished - Feb 10 2009
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry


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