The individuals with significantly reduced levels of carnitine in muscle cells suffer from weakness, even during moderate exercise, this is because due to low level of carnitine the oxidation of fatty acids does not takes place.
Human muscles contains a high level of carnitine. The carnitine transport the long chain fatty acids into the matrix of the mitochondria, which is a site of beta oxidation of fatty acids spares the muscle glycogen and delays the onset of fatigue.
In this condition, the muscle tissue cannot synthesize carnitine, thus it dependent on the blood for its carnitine transport. Blood carnitine is derived from endogenously biosynthesis in the liver and the kidney and exogenous sources in the diet.
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