Suberylglycine is a glycine-conjugated form of the dicarboxylic acid suberic acid.1 Urinary levels of suberylglycine are increased in patients with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency, an inborn error of fatty acid metabolism characterized by hypoketotic hypoglycemia, medium-chain dicarboxylic aciduria, and intolerance to fasting.2,3,4
1.Truscott, R.J., Hick, L., Pullin, C., et al.Dicarboxylic aciduria: The response to fastingClin. Chim. Acta94(1)31-39(1979) 2.Bhuiyan, A.K., Watmough, N.J., Turnbull, D.M., et al.A new simple screening method for the diagnosis of medium chain acyl-CoA dehydrogenase deficiencyClin. Chim. Acta165(1)39-44(1987) 3.Onkenhout, W., Venizelos, V., van der Poel, P.F.H., et al.Identification and quantification of intermediates of unsaturated fatty acid metabolism in plasma of patients with fatty acid oxidation disordersClin. Chem.41(10)1467-1474(1995) 4.Rinaldo, P., O'Shea, J.J., Coates, P.M., et al.Medium-chain acyl-CoA dehydrogenase deficiency. Diagnosis by stable-isotope dilution measurement of urinary n-hexanoylglycine and 3-phenylpropionylglycineN. Engl. J. Med.319(20)1308-1313(1988)