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

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.
Authors	Limei Zhang, Josef Lichtmannegger, Karl H Summer, Samuel Webb, Ingrid J Pickering, Graham N George
Journal	Biochemistry (Biochemistry) Vol. 48 Issue 5 Pg. 891-7 (Feb 10 2009) ISSN: 1520-4995 [Electronic] United States
PMID	19146437 (Publication Type: Comparative Study, Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, Non-P.H.S.)
Chemical References	Cation Transport Proteins Chelating Agents Copper Molybdenum tetrathiomolybdate Adenosine Triphosphatases Copper-Transporting ATPases
Topics	Adenosine Triphosphatases (deficiency, genetics) Animals Cation Transport Proteins (deficiency, genetics) Chelating Agents (administration & dosage, chemistry, metabolism) Copper (chemistry, metabolism) Copper-Transporting ATPases Disease Models, Animal Hepatolenticular Degeneration (diagnosis, drug therapy, enzymology) Kidney (metabolism, pathology) Liver (metabolism, pathology) Molybdenum (administration & dosage, chemistry, metabolism) Prospective Studies Rats Rats, Inbred LEC Rats, Mutant Strains

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