Isovalerylglycine is a byproduct of the catabolism of the amino acid leucine. Accumulation of isovalerylglycine occurs in Isovaleric Acidemia (IVA), an autosomal recessive disorder caused by mutations in the isovaleryl-CoA dehydrogenase gene. The deficiency of this enzyme in the metabolism of leucine leads to the accumulation of a series of isovaleryl-CoA metabolites, such as isovalerylglycine (IVG).
Storage Class Code
11 - Combustible Solids
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