3-Methylglutaconic Aciduria (3MGA)



3-Methylglutaconic aciduria (3MGA) is the name for a group of five different conditions. All 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. All variants of 3MGA are classified as organic acid conditions because it can result in a build-up of harmful amounts of organic acids and toxins in the body. 3MGA symptoms can include developmental delay, weak muscles, vision loss, frequent infections, delayed growth, and other complications.  If this condition is detected early and proper treatment is received, the severity of symptoms may be reduced.

Condition Type:



3-Methylglutaconic aciduria (3MGA) is a rare condition. Less than 20 cases of MGA type I have been reported. 3MGA type II (Barth syndrome) is estimated to affect 1 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 1 in every 10,000 individuals. Exact numbers of affected individuals with MGA type IV and MGA type V are not available. MGA type V has only been reported in the Hutterite population of North America and Canada.

More Information for Parents:
Also known as:
  • 3MGA
  • Barth syndrome (type II only)
  • Costeff optic atrophy syndrome (type III only)
  • Dilated cardiomyopathy with ataxia (type V only)
  • 3-methylglutaconyl CoA hydratase deficiency

Core Conditions