Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Trimethylaminuria


Other Names for this Disease
  • Fish malodor syndrome
  • Fish odor syndrome
  • Stale fish syndrome
  • TMAU
  • TMAuria
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Cause

Newline Maker

What causes trimethylaminuria?

Most cases of trimethylaminuria are caused by changes (mutations) in the FMO3 gene. This gene encodes an enzyme that is responsible for breaking down certain nitrogen-containing compounds such as trimethylamine. Trimethylamine is produced in the intestines when certain types of food (i.e. eggs, liver, legumes, fish and some vegetables) are digested. A mutation in this gene prevents the enzyme from working properly, so it is unable to break down compounds like trimethylamine. As trimethylamine begins to build up in the body, it is released in a person's sweat, urine, and breath. Because trimethylamine has a fishy odor, this leads to the characteristic features of trimethylaminuria.[1]
Last updated: 3/26/2015

References
  1. Trimethylaminuria. Genetics Home Reference. January 2013; http://ghr.nlm.nih.gov/condition=trimethylaminuria.


Other Names for this Disease
  • Fish malodor syndrome
  • Fish odor syndrome
  • Stale fish syndrome
  • TMAU
  • TMAuria
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.