4480 Ketoleucine Ketoleucine is a metabolite that accumulates in Maple Syrup Urine Disease (MSUD) and shown to compromise brain energy metabolism by blocking the respiratory chain; this is of relevance to the understanding of the pathophysiology of the neurological dysfunction of MSUD patients. (A3497). 816-66-0 70 C6H10O3 8 - 10°C 32 mg/mL Ketoleucine is a metabolite that accumulates in Maple Syrup Urine Disease (MSUD) and shown to compromise brain energy metabolism by blocking the respiratory chain. 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 keto-leucine are associated with Maple Syrup Urine Disease. 2014-08-29T06:51:13Z 2026-04-02T23:25:41Z C00233 48430 2K-4CH3-PENTANOATE DB03229 COI true CC(C)CC(=O)C(O)=O C6H10O3 InChI=1S/C6H10O3/c1-4(2)3-5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9) BKAJNAXTPSGJCU-UHFFFAOYSA-N 130.1418 130.062994186 Endogenous Liquid HMDB00695 CHEMBL445647 69 CHEM003386 DTXSID6061157 4-methyl-2-oxopentanoic acid