Trimethylaminuria (TMAU), also known as fish odor syndrome or fish malodor syndrome, is a rare metabolic disorder that causes a defect in the normal production of an enzyme named flavin-containing monooxygenase 3 (FMO3). When FMO3 is not working correctly or if not enough enzyme is produced, the body loses the ability to properly convert trimethylamine (TMA) from precursor compounds in food digestion into trimethylamine oxide (TMAO), through a process called N-oxidation. Trimethylamine then builds up and is released in the persons sweat, urine, and breath, giving off a strong fishy odor or strong body odor. Primary trimethylaminuria is caused by genetic mutations that affect the FMO3 function of the liver. A variant of TMAU (secondary trimethylaminuria or TMAU2) is an acquired form of TMAU where there is no genetic cause, yet excessive TMA is secreted. TMAU2 can be caused by a range of issues, including a precursor overload (ingesting too many precursors), hormonal issues related to menstrual cycles, liver damage, and liver and kidney failure. It is hypothesised that intestinal dysbiosis may cause temporary TMAU2 symptoms, however there is no direct evidence of this in a scientific study. Metabolic pathway
Trimethylamine enters the body via the consumption of certain foods and supplements. When food is consumed that contains TMA and/or TMAO (predominately seafood; saltwater fish, shellfish, seaweed and kelp), TMAO is converted by bacteria in the lower gastrointestinal tract (gut) into TMA.
Using slightly acidic detergent and body washes with a pH between 5.5 and 6.5Additionally, at least one study has suggested that daily intake of the supplements activated charcoal and copper chlorophyllin may temporarily improve the quality of life of individuals afflicted with TMAU by helping their bodies to oxidize and convert TMA to the odorless N-oxide (TMAO) metabolite. Study participants experienced subjective reduction in odor as well as objective reduction in TMA and increase in TMAO concentration measured in their urine. The study found that:

85% of test participants experienced complete loss of detectable "fishy" odor
10% experienced some reduction in detectable odor
5% did not experience any detectable odor reduction

History
The first clinical case of TMAU was described in 1970.

Notable media
In 2014, singer/songwriter Cassie Graves was first featured in the Daily Mail, the Daily Mirror, and The Metro UK newspapers in both print and Online, giving an interview about her experiences with Trimethylaminuria. The article was later repurposed in media across the globe, most notably by HuffPost.In 2016, Graves was then featured in Princess Productions Medical Mysteries on UKs Channel 5, which went on a journey to find an official diagnosis for the condition, and again sparked a global media interest in the condition. References
External links

This article incorporates public domain text from The U.S. National Library of Medicine and The National Human Genome Research Institute