Isovaleric Acidemia is treated with L-leucine restriction and supplemental glycine. Added glycine augments conversion of isovaleric acid (IVA) to isovalerylglycine (IVG) through glycine-N-acylase.

Isovaleric acidemia (IVA) is a rare genetic disorder that affects the metabolism of leucine, an essential amino acid. This condition is caused by a deficiency in the enzyme isovaleryl-CoA ...