3-Methylglutaric Acid

3-Methylglutaric acid is a metabolite of the essential amino acid L-leucine (Cay-34342). It is produced by hydrolysis of the L-leucine catabolic intermediate 3-methylglutaconyl-CoA (3-MG-CoA), which accumulates when the activity of 3-MG-CoA hydratase, the enzyme that converts 3-MG-CoA to HMG-CoA, is deficient, in the mitochondria. 3-Methylglutaric acid (5 mM) inhibits N+/K+-ATPase activity and induces the production of reactive oxygen species (ROS) in rat cortical synaptosomes, as well as induces lipid peroxidation in rat cortical supernatants. Urinary levels of 3-methylglutaric acid are increased in patients with 3-methylglutaconic aciduria type I, an inborn error of metabolism characterized by mutations in the gene encoding 3-MG-CoA hydratase that can lead to neurological impairments.Formal Name: 3-methyl-pentanedioic acid. CAS Number: 626-51-7. Synonyms: NSC 14870. Molecular Formula: C6H10O4. Formula Weight: 146.1. Purity: >95%. Formulation: (Request formulation change), A solid. Solubility: DMF: 30 mg/ml, DMSO: 30 mg/ml, Ethanol: 30 mg/ml, PBS (pH 7.2): 10 mg/ml. SMILES: O=C(CC(CC(O)=O)C)O. InChi Code: InChI=1S/C6H10O4/c1-4(2-5(7)8)3-6(9)10/h4H,2-3H2,1H3,(H,7,8)(H,9,10). InChi Key: XJMMNTGIMDZPMU-UHFFFAOYSA-N.