4394 Isobutyrylglycine Isobutyrylglycine is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. In certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. Acyl glycines are produced through the action of glycine N-acyltransferase (EC 2.3.1.13) which is an enzyme that catalyzes the chemical reaction:acyl-CoA + glycine < -- > CoA + N-acylglycineIsobutyrylglycine is identified in large amount in urine of patients with isobutyryl-CoA dehydrogenase deficiency. Isobutyryl-CoA dehydrogenase deficiency is a disorder caused by the deficiency of isobutyryl-CoA dehydrogenase that is involved in the catabolism of the branched-chain amino acid valine (A3426). 15926-18-8 10855600 C6H11NO3 White powder. 82.5°C No indication of carcinogenicity to humans (not listed by IARC). This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation. 2014-08-29T06:29:47Z 2026-04-03T00:41:23Z 70979 true CC(C)C(=O)NCC(O)=O C6H11NO3 InChI=1S/C6H11NO3/c1-4(2)6(10)7-3-5(8)9/h4H,3H2,1-2H3,(H,7,10)(H,8,9) DCICDMMXFIELDF-UHFFFAOYSA-N 145.1564 145.073893223 Endogenous Solid HMDB00730 CHEMBL2158191 9030891 Katz, J.; Lieberman, I.; Barker, H. A. Formation of propionyl-, butyryl-, and other acylglycines by enzymes of Clostridium kluyveri. Journal of Biological Chemistry (1953), 200 431-41. CHEM003300 DTXSID10445943 NS00015177 66.4 34.6486 14.569462707724016 3 3 2 4.0629876465018375 -1.8019866084514846 -1 0 -0.11 -0.91 1.79e+01 g/l