The role of carnitine in metabolism is very critical: it is a key component of the enzymes responsible for transporting long-chain FAs across the mitochondrial matrix where they can be used to produce energy. Carnitine is endogenously produced from the essential amino acids lysine and methionine in amounts that are sufficient to maintain homeostasis, when an individual's dietary intake includes a low to moderate amount of meat products. If there is a deficiency of carnitine, it is typically a result of impaired synthesis, increased degradation, inefficient transport, or abnormal renal handling. This can result in glucose dependency and perhaps even hypoglycemia. Skeletal musculature is weakened and may atrophy in addition to decreased myo-globin concentrations. Finally, cardiac muscle, which utilizes primarily FAs for fuel, may experience failure and frequent arrhythmias.


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