Lipids are the driving force for a longer period of exercise. Actually lipid metabolism takes over the role of fuel production from carbohydrates after initial bout of exercise for a sustained session of exercise. Carnitine palmitoyltransferase is the enzyme that is necessary for entry of appropriate lipids into mitochondria for further processing and energy production. Deficiency of this enzyme leads to failure in production of energy from lipid hence this condition is also known as carnitine palmitoyl transferase deficiency.
It usually presents in the young adults in the form of severe weakness after prolonged exercise.
Its symptoms include severe weakness after prolonged exercise. Myoglobinuria is present and more severe than that in glycogenoses. Respiratory compromise can occur to varying extent in advanced cases.