Causes And Risk Factors Of Trimethylaminuria

Trimethylaminuria (TMAU) is a genetic metabolic disease that renders the body incapable of breaking down a smelly compound called trimethylamine (TMA). In normal individuals, the body converts TMA into trimethylamine oxide (TMAO) through a complex enzyme system in the liver. Trimethylamine has an odor similar to rotting fish. When the liver enzyme is functioning properly, TMA is broken down into TMAO, which is odorless. However, individuals with trimethylaminuria lack this critical enzyme. These unfortunate individuals emit a body odor that smells like decomposing fish, garbage and urine. Trimethylaminuria is caused by a mutation to the gene known as flavin-containing monooxygenase 3 (FMO3).