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.

• Authors: W Lehnert
• Journal: Clinica chimica acta; international journal of clinical chemistry (Clin Chim Acta) Vol. 134 Issue 1-2 Pg. 207-12 (Oct 31 1983) ISSN: 0009-8981 [Print] Netherlands
• PMID: 6197208 (Publication Type: Journal Article)

Chemical References

• Hemiterpenes
• Pentanoic Acids
• Valerates
• isovaleric acid
• N-isovalerylalanine
• N-isovalerylsarcosine
• Alanine
• Sarcosine

Topics

• Alanine (analogs & derivatives, urine)
• Gas Chromatography-Mass Spectrometry (methods)
• Hemiterpenes
• Humans
• Pentanoic Acids (blood)
• Sarcosine (analogs & derivatives, urine)
• Valerates (blood)