Other Names for this Disease
- Fish odor syndrome
- Fish malodor syndrome
- Stale fish syndrome
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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.
Last updated: 3/26/2015
- Trimethylaminuria. Genetics Home Reference. January 2013; http://ghr.nlm.nih.gov/condition=trimethylaminuria.