4319 3-Methylglutaconic acid 3-Methylglutaconic acid is an intermediate (as the CoA thioester) in the leucine degradative pathway as well as the mevalonate shunt, a pathway that links isoprenoid metabolism with mitochondrial acetyl-CoA metabolism. 3-methylglutaconyl-CoA hydratase is involved in the metabolism process of 3-methylglutaconic acid. 3-Methylglutaconic acid accumulates in patients with a deficiency of this enzyme and a large amount of 3-methylglutaconic acid appear in urine. . (A3360). 5746-90-7 1551553 C6H8O4 White powder. Accumulation of 3-methylglutaconic acid in the body has been shown to be toxic. 3-methylglutaconyl-CoA hydratase is involved in the metabolism process of 3-methylglutaconic acid. 3-Methylglutaconic acid accumulates in patients with a deficiency of this enzyme and a large amount of 3-methylglutaconic acid appear in urine. (Wikipedia). No indication of carcinogenicity to humans (not listed by IARC). This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation. Chronically high levels of 3-methylglutaconic acid are associated with at least 5 inborn errors of metabolism including: 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency, 3-Methylglutaconic Aciduria Type I, 3-Methylglutaconic Aciduria Type III, 3-Methylglutaconic Aciduria Type IV and Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency). 2014-08-29T06:10:05Z 2026-04-06T13:39:15Z 3-Methylglutaconic acid 37245 true C\C(CC(O)=O)=C/C(O)=O C6H8O4 InChI=1S/C6H8O4/c1-4(2-5(7)8)3-6(9)10/h2H,3H2,1H3,(H,7,8)(H,9,10)/b4-2+ WKRBKYFIJPGYQC-DUXPYHPUSA-N 144.1253 144.042258744 Endogenous Solid HMDB00522 CHEMBL1401952 1267861 Aoyama, Takayuki; Kato, Kazuo; Shimada, Nobuyoshi; Fujii, Akio; Takita, Tomohisa. Production of 3-methylglutaconic acid. Jpn. Kokai Tokkyo Koho (1986), 3 pp. CHEM003225 NS00126341 74.6 33.5133 13.266891782956623 3 4 2 3.854151000940069 -2 0 0.37 -1.22 8.69e+00 g/l