Isovalerylglycine (IVG) is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. In certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation.
Isovalerylglycine is a byproduct of the catabolism of the aminoacid leucine.
Treatment consists of dietary protein restriction, particularly leucine.
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