4-Methyl-2-oxopentanoic acid

A metabolite of leucine catabolism

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货号: ajce46856
CAS: 816-66-0
别名: 4-甲基-2-氧代戊酸,α-Ketoisocaproic acid
分子式: C6H10O3
分子量: 130.14
纯度: >98%
溶解度: DMSO: ≥ 100 mg/mL (768.40 mM); Water: 100 mg/mL (768.40 mM)
储存: Store at -20°C
库存: 现货

Background

α-Ketoisocaproic acid is a metabolite of L-leucine catabolism.¹ 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 ?mol.² Urine levels of α-ketoisocaproic acid are reduced in db/db diabetic mice compared with heterozygous db/m non-diabetic mice.³ α-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 α-keto acid dehydrogenase (BCKAD) deficiency and leads to progressive ketoacidosis, failure to thrive, neurological dysfunction, and, potentially, death.²

1.Schiff, M., Ogier de Baulny, H., and Dionisi-Vici, C.Branched-chain organic acidurias/acidaemiasInborn metabolic diseases: Diagnosis and treatment277-294(2016) 2.Farias, H.R., Gabriel, J.R., Cecconi, M.L., et al.The metabolic effect of α-ketoisocaproic acid: In vivo and in vitro studiesMetab. Brain Dis.36(1)185-192(2021) 3.Kim, N.H., Hyeon, J.S., Kim, N.H., et al.Metabolic changes in urine and serum during progression of diabetic kidney disease in a mouse modelArch. Biochem. Biophys.64690-97(2018)