Used primarily in the treatment of hereditary tyrosinemia type 1 (HT-1), a rare genetic disorder. It works by inhibiting an enzyme involved in tyrosine metabolism, preventing the buildup of toxic metabolites that can cause severe liver and kidney damage. By controlling these metabolic byproducts, it helps improve survival and reduces the need for liver transplantation in affected patients. Also used in the management of alkaptonuria, where it helps reduce the production of a pigment-like substance that accumulates in connective tissues, slowing joint and organ damage. Its application has expanded into research for other metabolic conditions involving oxidative stress or abnormal protein deposition.