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Trimethylaminuria

Related Terms

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Background

  • Trimethylaminuria (TMAU) is an inherited metabolic disorder that is characterized by an offensive body odor that smells like rotting fish. This odor is due to the excessive excretion of a protein called trimethylamine (TMA) in the urine, sweat, and breath.
  • TMAU, also known as fish odor syndrome or fish malodor syndrome, is caused by defects in a protein called flavin-containing monooxygenase 3 (FMO3). The FMO3 protein is an enzyme produced by the liver that functions mainly to break down nitrogen-containing compounds.
  • TMA is consumed through the diet and is normally broken down through a process called N-oxygenation. The abnormal function of FMO3 results in the body's inability to break down TMA from food sources, especially choline-rich foods, such as milk, eggs, and peanuts. When this process does not work properly, TMA builds up and is released in the person's sweat, urine, and breath. This causes the characteristic smell associated with TMAU.
  • Trimethylaminuria is a rare genetic condition with unknown incidence. This inherited disorder demonstrates how genetics can alter the way dietary substances are broken down (metabolized). However, TMAU has been diagnosed in people who have liver disease but do not have a family history of the disorder.
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Risk Factors

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Causes

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Signs and Symptoms

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Types of the Disease

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Diagnosis

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Complications

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Treatment

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Integrative Therapies

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Prevention

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Author Information

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References

Natural Standard developed the above evidence-based information based on a thorough systematic review of the available scientific articles. For comprehensive information about alternative and complementary therapies on the professional level, go to www.naturalstandard.com. Selected references are listed below.

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The information in this monograph is intended for informational purposes only, and is meant to help users better understand health concerns. Information is based on review of scientific research data, historical practice patterns, and clinical experience. This information should not be interpreted as specific medical advice. Users should consult with a qualified healthcare provider for specific questions regarding therapies, diagnosis and/or health conditions, prior to making therapeutic decisions.