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Bioorg Med Chem Lett. 2012 Aug 1;22(15):4975-8. doi: 10.1016/j.bmcl.2012.06.024. Epub 2012 Jun 16.

γ-Butyrobetaine hydroxylase catalyses a Stevens type rearrangement.

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Department of Chemistry, University of Oxford, Chemistry Research Laboratory, Oxford, United Kingdom.


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 the synthesis of AMBA and confirm that it is a product of the BBOX catalysed oxidation of THP. AMBA reacts with formaldehyde, which is produced enzymatically by BBOX, to give a cyclic adduct.

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