Analyzing the Therapeutic Efficacy of Bis-Choline-Tetrathiomolybdate in the Atp7b-/- Copper Overload Mouse Model.

ABSTRACT: Bis-choline-tetrathiomolybdate, introduced as WTX101 (now known as ALXN1840), is a first-in-class copper-protein-binding agent for oral therapy of Wilson's disease. In contrast to other decoppering agents such as trientine or D-penicillamine it acts by forming a tripartite complex with copper and albumin, thereby detoxifying excess liver and blood copper through biliary excretion. Preclinical animal experimentation with this drug was typically done with the alternative ammonium salt of tetrathiomolybdate, which is expected to have identical properties in terms of copper binding. Here, we comparatively analyzed the therapeutic efficacy of ALXN1840, D-penicillamine and trientine in lowering hepatic copper content in Atp7b-/- mouse. Liver specimens were subjected to laser ablation inductively

SUBMITTER: Kim P 

PROVIDER: S-EPMC8698685 | biostudies-literature | 2021 Dec

REPOSITORIES: biostudies-literature