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Tetrathiomolybdate and Wilson’s Disease，2013 - I

Ammonium tetrathiomolybdate treats chronic Cu poisoning in sheep and is recommended for Wilson's disease in humans (congenital inability to excrete copper resulting in its accumulation) . In the tetrathiomolybdate-treated sheep Mo accumulated in brain, liver, kidney, heart, skeletal muscle, pituitary, adrenals, testes and ovaries and was retained after cessation of treatment, except in liver, kidney and skeletal muscle.

Cu increased and was retained in the cerebellum and medulla oblongata in the tetrathiomolybdate-treated high-Cu Cambridge groups. Brain Cu and Mo concentrations showed a strongly positive correlation in the high-Cu Ronaldsay group 7 months after tetrathiomolybdate treatment. Tetrathiomolybdate is not all excreted; Mo is widely distributed and retained in many organs including brain and pituitary. Tetrathiomolybdate may redistribute some displaced excess liver Cu to the brain.

The uptake of tetrathiomolybdate by the liver and the removal of copper accumulating in the liver in a form bound to metallothionein by tetrathiomolybdate were studied in Long-Evans cinnamon (LEC) rats, an animal model of Wilson’s disease, in order to develop better treatments for the disease and Cu toxicity  When the dose of tetrathiomolybdate is low, tetrathiomolybdate forms a complex with Cu that can be effluxed into the bloodstream, and then binds selectively to albumin.

When the dose is high, tetrathiomolybdate forms an insoluble complex, that is precipitated in the liver. Tetrathiomolybdate taken up by a cell is immobilized in the cell through the dose-dependent formation of a complex containing Cu, Mo and sulfur, which causes further uptake of tetrathiomolybdate. Tetrathiomolybdate does not remove Cu from ceruloplasmin.

Tetrathiomolybdate targets a cell accumulating excess Cu as Cu- metallothionein, and removes Cu selectively without interacting with Cu in Cu-enzymes. Tetrathiomolybdate is taken up by the liver depending on the amount of Cu accumulating in the form of metallothionein, and then Cu is effluxed together with Mo in the form of Cu/tetrathiomolybdate complex into the bloodstream.

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