描述
Ketoleucine, also known as alpha-ketoisocaproic acid or 2-oxoisocaproate, belongs to short-chain keto acids and derivatives class of compounds. Those are keto acids with an alkyl chain the contains less than 6 carbon atoms. Ketoleucine is slightly soluble (in water) and a weakly acidic compound (based on its pKa). Ketoleucine can be found in a number of food items such as arctic blackberry, sesame, sea-buckthornberry, and soft-necked garlic, which makes ketoleucine a potential biomarker for the consumption of these food products. Ketoleucine can be found primarily in most biofluids, including saliva, blood, cerebrospinal fluid (CSF), and urine, as well as in human muscle, neuron and prostate tissues. Ketoleucine exists in all living species, ranging from bacteria to humans. In humans, ketoleucine is involved in the valine, leucine and isoleucine degradation. Ketoleucine is also involved in several metabolic disorders, some of which include methylmalonate semialdehyde dehydrogenase deficiency, propionic acidemia, 3-methylglutaconic aciduria type IV, and 3-methylglutaconic aciduria type I. Ketoleucine is a non-carcinogenic (not listed by IARC) potentially toxic compound. Ketoleucine is a metabolite that accumulates in Maple Syrup Urine Disease (MSUD) and shown to compromise brain energy metabolism by blocking the respiratory chain (T3DB).
InChI标识符
InChI=1S/C6H10O3/c1-4(2)3-5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9)