2-Methylbutyrylglycine is a metabolite of the essential amino acid L-isoleucine.^1,2It is produced by hydrolysis of the L-isoleucine catabolic intermediate (S)-2-methylbutyryl-CoA, which accumulates when the activity of 2-methylbutyryl-CoA dehydrogenase is deficient. 2-Methylbutyrylglycine (0.5-5 mM) induces the formation of thiobarbituric acid reactive substances (TBARS) in isolated rat cerebral cortex.³Elevated levels of 2-methylbutyrylglycine are associated with 2-methylbutyryl-CoA dehydrogenase deficiency (2-MBCDD), also known as short/branched-chain acyl-CoA dehydrogenase (SBCAD) deficiency.^1,2

1.Gibson, K.M., Burlingame, T.G., Hogema, B., et al.2-Methylbutyryl-coenzyme A dehydrogenase deficiency: A new inborn error of L-isoleucine metabolismPediatr. Res.47(6)830-833(2000) 2.Fong, B.M.-W., Tam, S., and Leung, S.-Y.Quantification of acylglycines in human urine by HPLC electrospray ionization-tandem mass spectrometry and the establishment of pediatric reference interval in local ChineseTalanta88193-200(2012) 3.Knebel, L.A., Zanatta, Â., Tonin, A.M., et al.2-Methylbutyrylglycine induces lipid oxidative damage and decreases the antioxidant defenses in rat brainBrain Res.147874-82(2012)