3-methylglutaconic aciduria (3MGA) - Diseases and Conditions Identified in Children

3-methylglutaconic aciduria, type I (3MGA)


3-methylglutaconic aciduria, type I (3MGA) is an inborn error of metabolism, an inherited condition in which an enzyme, 3-methylglutaconyl-CoA hydratase, in the body is either missing or not working well. The job of this enzyme is to help break down the amino acid (a part of protein) known as leucine. 3MGA is an organic acid condition because it can lead to the build-up of harmful amounts of organic acids and toxins in the body. Symptoms can vary and may include developmental delay and/or other potential signs and symptoms. Support groups are available to help connect families living with 3MGA with others who have experience or expertise with this condition.

To learn more information about 3-methylglutaconic aciduria, visit Baby's First Test: 3-methylglutaconic aciduria and U.S. National Library of Medicine: 3-methylglutaconyl-CoA hydratase deficiency. A listing of contact information for Minnesota inborn errors of metabolism medical specialty providers/clinics serving children affected with or undergoing evaluation for conditions that can be detected through Minnesota Newborn Screening is available upon request.