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GTR Home > Conditions/Phenotypes > Trimethylaminuria

Summary

Excerpted from the GeneReview: Primary Trimethylaminuria
Primary trimethylaminuria is characterized by a fishy odor resembling that of rotten or decaying fish that results from excess excretion of trimethylamine in the urine, breath, sweat, and reproductive fluids. No physical symptoms are associated with trimethylaminuria. Affected individuals appear normal and healthy; however, the unpleasant odor often results in social and psychological problems. Symptoms are usually present from birth and may worsen during puberty. In females, symptoms are more severe just before and during menstruation, after taking oral contraceptives, and around the time of menopause.

Genes See tests for all associated and related genes

  • Also known as: FMOII, TMAU, dJ127D3.1, FMO3
    Summary: flavin containing dimethylaniline monoxygenase 3

Clinical features

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