Trimethylaminuria [Supplementary Concept]

A hereditary autosomal recessive condition which results from the abnormal presence of large amounts of volatile and malodorous trimethylamine within the body. This chemical, a tertiary aliphatic AMINE, is excreted in the urine, sweat (ichthyohidrosis), and breath, which take on the offensive odor of decaying fish. Mutations in the FMO3 gene have been identified. OMIM: 602079

Date introduced: August 25, 2010

MeSH Unique ID: C536561

Heading Mapped to:

• Metabolism, Inborn Errors
• Methylamines/urine

Entry Terms:

• Fish odor syndrome
• Fish malodor syndrome
• Fish-Odor Syndrome
• Stale Fish Syndrome
• TMAuria