γ-Butyrobetaine hydroxylase (BBOX) is a 2-oxoglutarate and Fe (II)-dependent oxygenase that catalyses the final step of l-carnitine biosynthesis in animals. BBOX catalyses the oxidation of 3-(2, 2, 2-trimethylhydrazinium) propionate (THP), a clinically used BBOX inhibitor, to form multiple products including 3-amino-4-(methyamino) butanoic acid (AMBA), which is proposed to be formed via a Stevens type rearrangement mechanism. We report ...