A comparison of the effects of penicillamine, trientine, and trithiomolybdate on [35S]-labeled metallothionein in vitro; implications for Wilson's disease therapy

J Inorg Biochem. 1991 Feb 1;41(2):87-92. doi: 10.1016/0162-0134(91)80002-y.

Abstract

The synthesis of radiolabeled metallothionein was induced in rats in vivo by the injection of CuSO4 and [35S]-cysteine. Treatment of "cold" rat liver cytosol "spiked" with purified [35S] metallothionein with Penicillamine and Trientine showed that even at relatively high concentrations (up to 50 mg/g liver, wet weight), these compounds had no effect on the copper peak or the position of the [35S] label in the cytosol eluate after Sephadex G-75 gel filtration. By contrast, incubation of the "spiked" liver cytosol with Trithiomolybdate, even at relatively low concentrations (0.5 mg/g liver, wet weight), resulted in a transfer of metallothionein copper to high molecular weight protein fractions; the position of the [35S] apoprotein was unaffected. This copper "stripping" effect on metallothionein supports clinical and other evidence that thiomolybdates have a genuine decoppering effect in vivo whereas Penicillamine and Trientine have another mode of action and indicates that thiomolybdates might provide a more rational alternate therapy for Wilson's disease patients.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Copper / metabolism
  • Cytosol / drug effects
  • Cytosol / metabolism
  • Hepatolenticular Degeneration / drug therapy*
  • Liver / drug effects
  • Liver / metabolism*
  • Male
  • Metallothionein / metabolism*
  • Molybdenum / pharmacology*
  • Penicillamine / pharmacology*
  • Rats
  • Rats, Inbred Strains
  • Sulfur Radioisotopes
  • Trientine / pharmacology*

Substances

  • Sulfur Radioisotopes
  • trithiomolybdic acid
  • Copper
  • Molybdenum
  • Metallothionein
  • Penicillamine
  • Trientine