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It is associated with a mild form of carnitine palmitoyl transferase I deficiency, putting homozygous individuals at risk for fasting hypoglycemia 9,10

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The authors suggest that the amount of Acetyl-CoA in the mitochondrion available for esterification of free carnitine was reduced in fasted or energy-restricted animals leading to increased tissue carnitine concentrations while acetylcarnitine levels were reduced [111]

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