What is Trimethylaminuria

Trimethylaminuria, also known as fish odor syndrome, is a rare metabolic disorder characterized by the inability to break down trimethylamine (TMA), a compound found in certain foods. This condition is caused by a deficiency in the enzyme flavin-containing monooxygenase 3 (FMO3), which is responsible for metabolizing TMA into an odorless substance. As a result, individuals with trimethylaminuria experience a strong and unpleasant fishy odor that is excreted through their breath, sweat, and urine.

Trimethylaminuria is typically inherited in an autosomal recessive manner, meaning that an affected individual must inherit two copies of the mutated gene, one from each parent. The condition can have a significant impact on a person's quality of life, leading to social isolation and psychological distress.

While there is currently no cure for trimethylaminuria, management strategies focus on minimizing symptoms. This may involve adopting a low-odor diet, avoiding certain foods rich in TMA precursors, and using activated charcoal or other supplements to reduce TMA levels in the body. Additionally, maintaining good personal hygiene practices can help mitigate the odor.

If you suspect you or someone you know may have trimethylaminuria, it is important to consult with a healthcare professional for proper diagnosis and guidance on managing the condition.