Cu-64 as a tool in Wilson‘s disease research

Ziel/Aim Wilson´s disease (WD) is a genetic disorder caused by a mutation of the copper-transporting ATPase (ATP7B). Excessive Cu-accumulation leads to severe liver-related symptoms that are conventionally treated with chelating agents such as d-penicillamine (D-PA). While being effective in restoring the Cu-homeostasis in early stages of WD, these treatments are accompanied by severe side effects and usually fail in progressed WD. It was already demonstrated that methanobactins (MBs), are able to restore Cu-homeostasis in vivo [1]. Here we used Cu-64 as a tool to assess copper depletion characteristics of HepG2 & ATP7b-KO HepG2 cells under treatment with native MB (OB3b) and modified MB (OB3b-N3) and compared the results with D-PA. In addition, MBs were labeled with Cu-64 to evaluate their uptake-kinetics.