Tiomolibdic acid salt, Bis-choline tetrathiomolybdate (ATN-224, WTX-101), is under investigation as a therapy against different cancers and Wilson’s disease (WD). ATN-224 is a second-generation analog of ammonium tetrathiomolybdate. ATN-224 is a novel copper chelator. ATN-224 inhibits CuZn superoxide dismutase 1 (SOD1) leading to antiangiogenic and antitumor effects. Strategically tailoring combination regimens that include ATN-224 and target ROS may be a viable approach to advance the treatment of melanoma. ATN-224 is in phase III clinical trial for the treatment of Hepatolenticular degeneration. WTX-101 is in phase II clinical trials for the treatment of Wilson's disease. Once daily WTX-101 treatment over 24 weeks improved neurologic disease, hepatic status and copper control in newly diagnosed WD patients. WTX-101 appears well tolerated. Drug-induced, paradoxical, neurological deterioration was not observed. This compound has received orphan drug designation in both the United States and the European Union. WTX-101 was originally discovered by University of Michigan and now is being developed by Wilson Therapeutics by acquisition.