C16 Carnitine has a potential to inhibit glycine transporter 2 (GlyT2). It acts as a key energy donor for β-oxidation. Palmitoyl carnitine activates the Ca2+ release channel of skeletal muscle sarcoplasmic reticulum. Elevated levels of palmitoyl L-carnitine due to palmitoyl transferase II deficiency causes autosomal recessive disorder of energy metabolism, which primarily affects skeletal muscle.