Sigma-Aldrich SODIUM 4-METHYL-2-OXOVALERATE LEUCINE

α-Ketoisocaproic acid is a leucine metabolite that is known to induce insulin secretion from the βcells of pancreas.[2] Enteral infusion of aα-Ketoisocaproate is found to ameliorate, endotoxemic condition. The resulting ketone bodies obtained from its conversion, might serve as an energy source.[3] Accumulation of α-Ketoisocaproic acid is characterized in branched chain ketoaciduria, which is caused due to the lack of branched chain L-2-keto acid dehydrogenase activity.[4][5] α-Ketoisocaproic acid causes dissociation in the oxidative phosphorylation reaction and acts as a metabolic inhibitor of α-ketoglutarate dehydrogenase. Thus, leads to a defect in the mitochondrial homeostasis, observed in branched chain ketoaciduria.[5]