Description_x000D_ Application_x000D_ Sodium 4-methyl-2-oxovalerate has been used for quantifying branched-chain keto acids from cell extracts by HPLC-fluorescence method._x000D_ Packaging_x000D_ 1, 5 g in poly bottle_x000D_ Biochem/physiol Actions_x000D_ α-Ketoisocaproic acid is a leucine metabolite that is known to induce insulin secretion from the βcells of pancreas. 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. 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. 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.