Trimethylaminuria: the fish malodor syndrome

Drug Metab Dispos. 2001 Apr;29(4 Pt 2):517-21.


The fish malodor syndrome (also known as the fish odor syndrome and trimethylaminuria) is a metabolic disorder characterized by the presence of abnormal amounts of the dietary-derived tertiary amine, trimethylamine, in the urine, sweat, expired air, and other bodily secretions. Trimethylamine itself has the powerful aroma of rotting fish, and this confers upon the sufferer a highly objectionable body odor, which can be destructive to the personal, social, and work life of the affected individual. In recent years, much progress has been made at all levels-clinical, epidemiological, biochemical, and genetic-in our understanding of this unfortunate condition. The present article summarizes this progress, draws attention to the different types of fish malodor syndrome, and highlights the current needs in the treatment of such patients.

Publication types

  • Congress

MeSH terms

  • Humans
  • Metabolism, Inborn Errors / diagnosis
  • Metabolism, Inborn Errors / genetics
  • Metabolism, Inborn Errors / metabolism*
  • Metabolism, Inborn Errors / therapy
  • Methylamines / urine*
  • Oxidation-Reduction
  • Polymorphism, Genetic


  • Methylamines
  • trimethylamine