This loss frequently leads to carnitine deficiency if supplemental carnitine is not provided. If carnitine concentrations are inadequate, entry of fatty acids into the mitochondria are blocked. In addition, scavenging of the toxic OA compounds is impaired leading to a buildup inside the mitochondria. We receive a significant amount of carnitine from foods we eat. Our bodies can also synthesize carnitine from proteins we eat.
Newborns have less synthetic capacity to make carnitine than children and adults.
Carnitine has been added to most infant formulas and is also found in mothers' breast milk. Carnitine is supplemented in many of the special formulas designed to treat organic acidemias. But usually additional carnitine, administered separately, is recommended in the treatment of some of the fatty acid disorders. Especially during times of illness and metabolic stress, the defective enzyme results in a buildup of the toxic OA compounds. These compounds bind to carnitine in the mitochondria and are then excreted in the urine.