This report describes general characteristics of the group of diseases classified as 3-methylglutaconic aciduria. 3-methylglutaconic aciduria is a genetically heterogeneous disorder, with multiple implicated genes and mapped loci. A list of 3-methylglutaconic aciduria subtypes, as defined by OMIM, can be found by following the link in the "OMIM phenotypic series" section, below. The table below includes links to more detailed reports for subtypes that have been investigated using fly models.
[updated Jun. 2017 by FlyBase; FBrf0222196]
Methylglutaconic aciduria is a clinically and genetically heterogeneous disorder whose metabolic landmark is urinary excretion of 3-methylglutaconic acid (3-MGA).