title: N-Isovalerylalanine and N-isovalerylsarcosine: two new minor metabolites in isovaleric acidemia.
abstract: Besides N-isovalerylglycine, isovaleric, 3-hydroxyisovaleric, 4-hydroxyisovaleric, methylsuccinic, mesaconic, 3-hydroxyisoheptanoic and N-isovalerylglutamic acids the excretion of hitherto unreported N-isovalerylalanine and N-isovalerylsarcosine, two minor but characteristic constituents of the organic acid profile in isovaleric acidemia, is described. The new metabolites are assumed to be formed from isovaleryl-CoA by action of the enzyme glycine N-acylase on alanine and sarcosine, respectively.
mesh_terms: D000409:Alanine / Q000031:analogs & derivatives* / Q000652:urine; D008401:Gas Chromatography-Mass Spectrometry / Q000379:methods; D045782:Hemiterpenes; D006801:Humans; D010421:Pentanoic Acids / Q000097:blood*; D012521:Sarcosine / Q000031:analogs & derivatives* / Q000652:urine; D014631:Valerates / Q000097:blood*
chemical_list: D045782:Hemiterpenes; D010421:Pentanoic Acids; D014631:Valerates; C008216:isovaleric acid; C039949:N-isovalerylalanine; C039948:N-isovalerylsarcosine; D000409:Alanine; D012521:Sarcosine