3-methylglutaconic aciduria (3MGA)


3-methylglutaconic aciduria (3MGA) is the name for a group of five different conditions. All of these conditions affect the body’s mitochondria, the part of a human cell that produces energy. The causes, symptoms, and treatment of the five different types of 3MGA vary and have distinct signs and symptoms. All types of 3MGA are organic acid conditions because they can result in a build-up of harmful amounts of organic acids and toxins in the body. All types will require monitoring by a cardiologist.

These conditions are known as: 3-MGA type I (methylglutaconyl CoA hydratase deficiency), 3-MGA-type II (Barth syndrome), 3-MGA type III (Costeff optic atrophy syndrome), 3-MGA type IV, 3-MGA type V (Dilated cardiomyopathy with ataxia).

To learn more information about 3-methylglutaconic aciduria, visit Baby's First Test: 3-methylglutaconic aciduria and U.S. National Library of Medicine: 3-methylglutaconic aciduria. Please see Medically Prescribed Formula for information about medically prescribed formula and pharmacologic interventions for inborn errors of metabolism. Visit Board Certified Metabolic Providers (PDF: 2 pages/204KB) for a listing of medical specialists and clinic contact information.