Nutritional Disorders Chapter 2. Carnitine palmitoyltransferase catalyzes transesterification of the fatty acyl group from CoA to carnitine, which is then transported through the inner membrane of the mitochondrion. A second transesterification inside the mitochondrion regenerates fatty acyl CoA for -oxidation.
Animal foods are rich in carnitine because animal cells can synthesize carnitine.
The human requirement for carnitine is thus met by a combination of endogenous biosynthesis and dietary intake. Carnitine deficiency may cause muscle necrosis, myoglobinuria, lipid-storage myopathy, hypoglycemia, fatty liver, and hyperammonemia with muscle ache, fatigue, and confusion. 6 h. Diminished synthesis caused by severe liver disease may lower body carnitine levels as may excessive loss due to dialysis in chronic renal failure. Hypoglycemia and skeletal muscle weakness, associated with biochemical evidence of carnitine deficiency during long-term TPN, have been reversed by carnitine therapy. All rights reserved.