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IVA occurs when an enzyme, called “isovaleryl-CoA dehydrogenase”, is either missing or not working properly. This enzyme’s job is to help break down a substance called “isovaleryl-CoA”. It is made in the body when the amino acid leucine is broken down. When a child with IVA eats food containing leucine, a substance called isovaleric acid builds up in the blood and causes problems. Leucine is found in all foods that contain protein.