Trimethylaminuria (Fish-Odor Syndrome)

Arseculeratne, Gehan; Wong, Alvin K. C.; Goudie, David; Ferguson, James

doi:10.1001/archderm.143.1.81

Cited by 20 publications

(11 citation statements)

References 52 publications

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“…1 In addition, marine fish contain large amounts of trimethylamine-N-oxide (TMAO) (which is believed to have antifreeze properties), which can be converted back to trimethylamine by gut bacteria. 2 Primary TMAU is most often caused by a functional defect of FMO3 (Fig. 1), 3 and the genetic disorder is inherited in an autosomal recessive manner as a consequence of mutations in the FMO3 gene.…”

Section: Legged Like a Man And His Fins Like Arms! Warm O' My Troth! mentioning

confidence: 99%

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Trimethylaminuria: An under‐recognised and socially debilitating metabolic disorder

Christodoulou

2011

J Paediatrics Child Health

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Primary flavin mono-oxygenase 3 deficiency, an inborn error of choline metabolism, leads to an accumulation of trimethylamine, which because of its associated pungent odour of rotting fish, is a socially crippling disorder. Although it often has its onset in early childhood, it may take years or even decades before the diagnosis is established. In this review the clinical biochemical and genetic features of the disorder are reported. The principles of therapy will also be covered, including dietary, pharmacological approaches, as well as techniques used to manipulate the gastrointestinal environment as a strategy to reduce the gastrointestinal load of trimethylamine.

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Section: Legged Like a Man And His Fins Like Arms! Warm O' My Troth! mentioning

confidence: 99%

“…The odour becomes more prominent during periods of stress, with fever and with strenuous exercise as a consequence of increased sweating 1 . In addition, marine fish contain large amounts of trimethylamine‐N‐oxide (TMAO) (which is believed to have antifreeze properties), which can be converted back to trimethylamine by gut bacteria 2 …”

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confidence: 99%

Trimethylaminuria: An under‐recognised and socially debilitating metabolic disorder

Christodoulou

2011

J Paediatrics Child Health

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“…While early reports of TMAO are intriguing, further study is required. 11,12 M A N U S C R I P T A C C E P T E D ACCEPTED MANUSCRIPT 7 We collected fasting serum samples in 1286 individuals enrolled in a multiethnic populationbased study of adults, some of whom had established CVD, in Canada (SHARE and SHARE-AP). 13,14 Using a nested case-control approach, in 292 consecutive individuals (99 CVD cases and 193 unmatched controls), we first assessed the stability of TMAO and L-carnitine in stored samples after repeated freeze-thaw cycles, and then investigated the association of TMAO and carnitine with CVD and intimal medial thickness (IMT) in this population-based cohort.…”

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confidence: 99%

The Relationship Between Trimethylamine-N-Oxide and Prevalent Cardiovascular Disease in a Multiethnic Population Living in Canada

Mente¹,

Chalcraft²,

Ak³

et al. 2015

Canadian Journal of Cardiology

114

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“…Deficiency of the enzyme results in excessive excretion of trimethylamine in urine, which generates a foul body odor redolent of rotten fish. This may have significant social and psychosocial ramifications [1]. Persistent (primary) trimethylaminuria in children is caused by an autosomal-recessively inherited mutation in the FMO3 gene.…”

Section: Discussionmentioning

confidence: 99%