– BBOX1, also known as gamma-BBH, belongs to thegamma-BBH/TMLD family. It is highly expressed in kidney and moderately expressed in liver. BBOX1 catalyzes the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthetic pathway. Carnitine is essential for the transport of activated fatty acids across the mitochondrial membrane during mitochondrial beta-oxidation. BBOX1 is an inhibition target for mildronate which can be used to treatanginaandmyocardial infarction. Mildronate may also be beneficial for the treatment ofneurological disorder,diabetes, andseizures andalcohol intoxication.