alpha-Ketoisocaproic Acid

alpha-Ketoisocaproic acid is a metabolite of L-leucine (Cay-34342) catabolism. It increases the production of reactive species and decreases the activity of mitochondrial complex I, also known as NADH dehydrogenase, and complex II-III in the rat hippocampus when administered at an intracerebroventricular dose of 4 µmol. Urine levels of alpha-ketoisocaproic acid are reduced in db/db diabetic mice compared with heterozygous db/m non-diabetic mice. alpha-Ketoisocaproic acid accumulates in the tissues and body fluids of patients with maple syrup urine disease, an inborn error of metabolism characterized by branched-chain alpha-keto acid dehydrogenase (BCKAD) deficiency and leads to progressive ketoacidosis, failure to thrive, neurological dysfunction, and, potentially, death.Formal Name: 4-methyl-2-oxo-pentanoic acid. CAS Number: 816-66-0. Synonyms: 2-Ketoisocaproate, alpha-Ketoisocaproate, 2-Ketoisocapronic Acid, KIC, 4-MOP, 2-Oxoisocaproic Acid, 4-methyl-2-Oxopentanoate, 4-methyl-2-Oxovaleric Acid, 4-methyl-2-Oxovalerate. Molecular Formula: C6H10O3. Formula Weight: 130.1. Purity: >95%. Formulation: (Request formulation change), A liquid. Solubility: DMF: 30 mg/ml, DMSO: 30 mg/ml, Ethanol: 30 mg/ml. lambdamax: 241 nm. SMILES: O=C(C(CC(C)C)=O)O. InChi Code: InChI=1S/C6H10O3/c1-4(2)3-5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9). InChi Key: BKAJNAXTPSGJCU-UHFFFAOYSA-N.