Of particular interest is an adventitious reaction catalyzed by γ‐butyrobetaine hydroxylase (BBOX), which normally performs a standard hydroxylation reaction during carnitine biosynthesis.[17] When incubated with trimethylhydrazine‐propionate (THP), a BBOX inhibitor used to treat cardiovascular disease, this enzyme catalyzes a DfmD‐like rearrangement coupled with formaldehyde release. This evidence concerns the gene BBOX1 and cardiovascular disorder.