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Type: Journal article
Title: Tetrathiomolybdate causes formation of hepatic copper-molybdenum clusters in an animal model of Wilson's disease
Author: George, G.
Pickering, I.
Harris, H.
Gailer, J.
Klein, D.
Lichtmannegger, J.
Summer, K.
Citation: Journal of the American Chemical Society, 2003; 125(7):1704-1705
Publisher: Amer Chemical Soc
Issue Date: 2003
ISSN: 0002-7863
Statement of
Graham N. George, Ingrid J. Pickering, Hugh H. Harris, Jürgen Gailer, Dominik Klein, Josef Lichtmannegger, and Karl-Heinz Summer
Abstract: Wilson's disease is an autosomal recessive human illness in which large quantities of copper accumulate in various organs, including the brain and the liver. If left untreated, it results in hepatitis, neurological complications, and death. Long-Evans Cinnamon (LEC) rats have a homologous mutation to Wilson's disease and thus provide an animal model. Liver lysosomes from tetrathiomolybdate-treated LEC rats were isolated and analyzed by Cu and Mo K-edge X-ray absorption spectroscopy. The lysosomes contained a Cu-Mo-S cluster in which the Mo is coordinated by four sulfurs at 2.24 A with approximately three copper neighbors at 2.70 A. Each Cu is coordinated to 3-4 sulfurs at 2.28 A with approximately one Mo neighbor at 2.70 A. These results indicate the formation of a biologically novel molybdenum-copper-sulfur cluster.
Keywords: Liver; Lysosomes; Animals; Rats, Inbred LEC; Humans; Rats; Hepatolenticular Degeneration; Disease Models, Animal; Sulfur; Copper; Molybdenum; Spectrometry, X-Ray Emission; Molecular Structure; Fourier Analysis
Description: Copyright © 2003 American Chemical Society
RMID: 0020081445
DOI: 10.1021/ja029054u
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Appears in Collections:Chemistry and Physics publications
Environment Institute publications

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