A review of trimethylaminuria: (fish odor syndrome)
- PMID: 24307925
- PMCID: PMC3848652
A review of trimethylaminuria: (fish odor syndrome)
Abstract
Trimethylaminuria, better known as fish odor syndrome, is a psychologically disabling condition in which a patient emits a foul odor, which resembles that of rotting fish. The disorder is most commonly caused by an inherited deficiency in flavin monooxygenase 3, the vital enzyme for the metabolism of trimethylamine, which is the compound responsible for the unpleasant odor. The condition is uncommon, but there has been recent research to suggest that the diagnosis may often be overlooked. Moreover, it is important to be cognizant of this condition because there are reliable diagnostic tests and the disorder can be devastating from a psychosocial perspective. While there is no cure, many simple treatment options exist that may drastically improve the quality of life of these patients. This article will review the literature with an emphasis on the psychosocial impact and treatment options.
Similar articles
-
Primary trimethylaminuria (fish odor syndrome) and hypothyroidism in an adolescent.Turk J Pediatr. 2017;59(5):614-616. doi: 10.24953/turkjped.2017.05.020. Turk J Pediatr. 2017. PMID: 29745129
-
Biochemical and clinical aspects of the human flavin-containing monooxygenase form 3 (FMO3) related to trimethylaminuria.Curr Drug Metab. 2003 Apr;4(2):151-70. doi: 10.2174/1389200033489505. Curr Drug Metab. 2003. PMID: 12678693 Review.
-
[Fish odor syndrome: A socially disabling disorder].Rev Med Interne. 2022 Mar;43(3):178-180. doi: 10.1016/j.revmed.2021.12.010. Epub 2022 Jan 7. Rev Med Interne. 2022. PMID: 35012788 French.
-
A compound heterozygous mutation in the FMO3 gene: the first pediatric case causes fish odor syndrome in Korea.Korean J Pediatr. 2017 Mar;60(3):94-97. doi: 10.3345/kjp.2017.60.3.94. Epub 2017 Mar 27. Korean J Pediatr. 2017. PMID: 28392825 Free PMC article.
-
Trimethylaminuria in a girl with Prader-Willi syndrome and del(15)(q11q13).Am J Med Genet. 1993 Feb 1;45(3):335-9. doi: 10.1002/ajmg.1320450310. Am J Med Genet. 1993. PMID: 8434620 Review.
Cited by
-
FMO3 deficiency of duck leads to decreased lipid deposition and increased antibacterial activity.J Anim Sci Biotechnol. 2022 Nov 16;13(1):119. doi: 10.1186/s40104-022-00777-1. J Anim Sci Biotechnol. 2022. PMID: 36380386 Free PMC article.
-
Trimethylamine modulates dauer formation, neurodegeneration, and lifespan through tyra-3/daf-11 signaling in Caenorhabditis elegans.Aging Cell. 2021 May;20(5):e13351. doi: 10.1111/acel.13351. Epub 2021 Apr 5. Aging Cell. 2021. PMID: 33819374 Free PMC article.
-
[Metabonomics studies of urine from APP/PS1 mice with early-stage Alzheimer's disease].Zhejiang Da Xue Xue Bao Yi Xue Ban. 2018 Dec 25;47(6):636-642. doi: 10.3785/j.issn.1008-9292.2018.12.12. Zhejiang Da Xue Xue Bao Yi Xue Ban. 2018. PMID: 30900843 Free PMC article. Chinese.
-
Impact of trimethylaminuria on daily psychosocial functioning.JIMD Rep. 2020 Oct 6;57(1):67-75. doi: 10.1002/jmd2.12170. eCollection 2021 Jan. JIMD Rep. 2020. PMID: 33473342 Free PMC article.
-
Serum trimethylamine N-oxide levels among coronary artery disease and acute coronary syndrome patients: a systematic review and meta-analysis.Ann Med Surg (Lond). 2023 Nov 8;85(12):6123-6133. doi: 10.1097/MS9.0000000000001426. eCollection 2023 Dec. Ann Med Surg (Lond). 2023. PMID: 38098555 Free PMC article. Review.
References
-
- Treacy EP, Akerman BR, Chow LM, et al. Mutations of the flavin-containing monooxygenase gene (FMO3) cause trimethylaminuria, a defect in detoxification. Hum Mol Genet. 1998;7:839–845. - PubMed
-
- Ziegler DM. Flavin-containing monooxygenases: catalytic mechanism and substrate specificities. Drug Metab Rev. 1988;19:1–32. - PubMed
-
- Jones KC, Ballou DP. Reactions of the 4a-hydroperoxide of liver microsomal flavin-containing monooxygenase with nucleophilic and electrophilic substrates. J Biol Chem. 1986;261:2553–2559. - PubMed
-
- Humbert JA, Hammond KB, Hathaway WE, et al. Trimethylaminuria: the fish-odour syndrome. Lancet. 1970;2:770–771. - PubMed
-
- Mitchell SC, Smith RL. Trimethylaminuria: the fish malodor syndrome. Drug Metab Dispos. 2001;29:517–521. - PubMed
Publication types
LinkOut - more resources
Full Text Sources