Concept

Trimethylaminuria

Summary
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 person's sweat, urine, and breath, giving off a fishy odor. Primary trimethylaminuria is caused by genetic mutations that affect the FMO3 function of the liver. Symptoms matching TMAU can also occur when there is no genetic cause, yet excessive TMA is excreted - this has been described as secondary trimethylaminuria (TMAU2). TMAU2 can be caused by a precursor overload (ingesting too many dietary TMA precursors), hormonal issues related to menstrual cycles, liver damage, or liver and kidney failure. As a symptom rather than a disease, TMAU2 is temporary and will resolve as the underlying cause is remedied (in the instance of menstrual related TMAU2, at menopause). 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. When a food substance, supplement or medicine that contains a TMA precursor (choline or carnitine) is ingested. Some precursor is absorbed into the bloodstream in the small intestine before reaching the gut (the RDI of choline is 450–550 mg per day, which is absorbed this way), however there is a limit to the transport capacity of the intestine, and not all precursor is exposed to the process. Unabsorbed precursor ends up in the gut.
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