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Description
First, in the cytosol, VPA is activated and links with reduced acetyl coenzyme A (CoA-SH) to form valproyl-CoA (by the ATP-dependent medium-chain acyl-CoA synthetase, located on the outer side of the mitochondrial membrane)
When taken in doses that do not exceed 2g per day, there is very little risk of L carnitine supplementation creating any side effects
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Individuals with CPT I deficiency are at risk for nervous system damage, liver failure, coma, and sudden death
