BBOX1, also known as gamma-BBH, belongs to the gamma-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 treat angina andmyocardial infarction. Mildronate may also be beneficial for the treatment ofneurological disorder, diabetes, and seizures and alcohol intoxication.