Ella-May Hards

1 Introduction ʟ-carnitine and its acyl derivatives are essential for a variety of biological functions and metabolic pathways,[1-3] the most important of which is the transport of fatty acids into the mitochondria for β-oxidation, producing ATP (Figure 1A).[1, 4] This process, known as ‘the carnitine shuttle’, proceeds via conjugation of fatty acids to carnitine by carnitine palmitoyltransferase (CPT1) on the outer mitochondrial membrane to generate acyl-carnitines.