| Identification | Back Directory | [Name]
(E)-3-methylpent-2-enedioic acid | [CAS]
372-42-9 | [Synonyms]
(E)-3-Methylpentenedioic acid
(E)-3-methylpent-2-enedioic acid
(2E)-3-methylpent-2-enedioic acid | [Molecular Formula]
C6H8O4 | [MDL Number]
MFCD01556044 | [MOL File]
372-42-9.mol | [Molecular Weight]
144.125 |
| Hazard Information | Back Directory | [Uses]
clinical testing | [Definition]
ChEBI: 3-methylglutaconic acid is a methyl-branched fatty acid. | [Biological Activity]
3-Methylglutaconic aciduria (MGCA) is a group of metabolic disorders characterized by increased urinary excretion of 3-methylglutaconic acid and 3-methylglutaric acid. Five distinct forms of MGCA have been recognized. MGCA type 1 is caused by primary deficiency of the mitochondrial enzyme 3-methylglutaconyl-CoA hydratase (3-MGCH)resulting in a block of leucine degradation. In all other typesthe activities of 3-MGCH and other enzymes of leucine degradation are normal and the MGCA is thought to be secondary to a defect in another pathway. MGCA type 2also known as Barth syndromeis an X-linked cardiomyopathy associated with skeletal myopathyneutropeniaand growth retardation. MGCA type 3also referred to as Costeff optic atrophy syndromeis an autosomal recessive disorder caused by mutations in the gene OPA3. It is characterized by early-onset bilateral optic atrophylater-onset extrapyramidal dysfunction. MGCA type 5 caused by mutation in the DNAJC19 geneis characterized |
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