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Article

Diagnosis and management of trimethylaminuria (FMO3 deficiency) in children.

Journal of inherited metabolic disease - 1 Feb 2006

Chalmers R A, Bain M D, Michelakakis H, Zschocke J, Iles R A

Abstract excerpt

Persistent trimethylaminuria in children is caused by autosomal recessively inherited impairment of hepatic trimethylamine (TMA) oxidation due to deficiency of flavin monooxygenase 3 (FMO3) secondary to mutations in the FMO3 gene. Trimethylaminuria or 'fish odour syndrome' is due to excessive excretion into body fluids and breath of TMA derived from the enterobacterial metabolism of dietary precursors. The...

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Source

Identifiers

PMID: 16601883
DOI: 10.1007/s10545-006-0158-6

Genes

FMO3

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

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Diagnosis and management of trimethylaminuria (FMO3 deficiency) in children.PMID 16601883Read ↗

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