(3-MGA) 3-Methylglutaconic acidemia (MGA) or 3-Methylglutaconyl-CoA Hydratase Deficiency

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. However, all types of 3MGA are classified as organic acid conditions because they can result in a build-up of harmful amounts of organic acids and toxins in the body.

3-methylglutaconic aciduria (3-MGA) is considered a rare condition. Less than 20 cases of MGA type I have been reported. 3MGA type II is estimated to affect one out of every 200,000 males. MGA type III is extremely rare in most populations. It is more common in the Iraqi-Jewish population where it affects approximately one in every 10,000 individuals. Exact numbers of affected individuals with MGA type IV and MGA type V are currently not available. MGA type V has only been reported in the Hutterite population of North America and Canada.

Also known as: 3-MGA, Barth syndrome (Type II only), Costeff optic atrophy syndrome (Type III only), Dilated cardiomyopathy with ataxia (Type V only), 3-methylglutaconyl CoA hydratase deficiency​.