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Potential use of tetrathiomolybdate in copper storage diseases.

Abstract Tetrathiomolybdate (TTM) is an effective decoppering agent. The mechanism of liver copper (Cu) removal by TTM was evaluated, using radiolabelled 67Cu, and 99Mo labelled TTM, with special reference to the relative contribution by the different excretory pathways in liver Cu removal. TTM increased the excretion of Cu via bile, urine and faeces. The increased biliary Cu excretion is relevant, as most of the important Cu storage diseases in man and animals are the result of the inability of the hepatocytes to excrete excess Cu into bile.