Home > D. Systemic pathology > Genetic and developmental anomalies > Genetic metabolic diseases > trimethylaminuria
trimethylaminuria
Friday 1 August 2008
Definition: Trimethylaminuria (TMAU),is a rare metabolic disorder that causes a defect in the normal production of the enzyme 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 breakdown trimethylamine (TMA) from precursor compounds in food into trimethylamine oxide (TMAO) through a process called N-oxygenation.
Trimethylamine then builds up and is released in the person’s sweat, urine, and breath, giving off a strong fishy odor.