Uses
3-Methylglutaconic acid is a glutarate which builds up in the urine in 3-methylglutaconic aciduria. 3-methylglutaconic aciduria is a term used to describe five different disorders that impair the functioning of energy-producing centers within cells (mitochondria)
Definition
ChEBI: A dicarboxylic acid comprising (E)-glutaconic acid carrying a 3-methyl substituent.
Biological Activity
3-Methylglutaconic acid is a metabolite (as the CoA thioester) in the leucine degradative pathway as well as the mevalonate shunt, a pathway th at links isoprenoid metabolism with mitochondrial acetyl-CoA metabolism. 3-Methylglutaconic acid accumulates in patients with a deficiency of 3-methylglutaconyl-CoA hydratase.
