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Open Access Green as soon as Postprint is submitted to ZB.
Tracing copper-thiomolybdate complexes in a prospective treatment for Wilson's disease.
Biochemistry 48, 891-897 (2009)
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.
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Publication type
Article: Journal article
Document type
Scientific Article
Keywords
ray-absorption spectroscopy; cuprous-thiolate clusters; ammonium tetrathiomolybdate; polyatomic-molecules; transporting atpase; initial therapy; animal-model; lec rats; gene; liver
ISSN (print) / ISBN
0006-2960
e-ISSN
1520-4995
Journal
Biochemistry
Quellenangaben
Volume: 48,
Issue: 5,
Pages: 891-897
Publisher
American Chemical Society (ACS)
Non-patent literature
Publications
Reviewing status
Peer reviewed