Diseases & Conditions


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Trimethylaminuria


Synonyms of Trimethylaminuria
  • Fish Odor Syndrome
  • Flavin Containing Monooxygenase 3
  • FMO3
  • FMO, Adult Liver Form
  • Stale Fish Syndrome

Disorder Subdivisions



    General Discussion
    Trimethylaminuria is a rare disorder in which the body?s metabolic processes fail to alter the chemical trimethylamine. Trimethylamine is notable for its unpleasant smell. It is the chemical that gives rotten fish a bad smell. When the normal metabolic process fails, trimethylamine accumulates in the body, and its odor is detected in the person?s sweat, urine and breath. The consequences of emitting a foul odor can be socially and psychologically damaging among adolescents and adults. The genetic or primary form of this disorder is transmitted as an autosomal recessive trait.

    A secondary form of trimethylaminuria may result from the side effects of treatment with large doses of the amino acid derivative L-carnitine (levocarnitine). The metabolic deficiency occurs as a result of a failure in the cell to make a specific protein, in this case, the enzyme flavin-containing monooxygenase3. Enzymes are nature?s catalysts and act to speed up biochemical activities. Without this enzyme, foods containing carnitine, choline and/or trimethylamine-N-oxide are processed to trimethylamine and no further, causing a strong fishy odor. This secondary form of the disorder is a result of an overload of trimethylamine. In this case, there is not enough of the enzyme to get rid of the excess trimethylamine.

    Organizations related to Trimethylaminuria
    • CLIMB (Children Living with Inherited Metabolic Diseases)
      Climb Building
      Crewe Intl CW2 6BG
      Phone #: +44- 87-0 7700 325
      800 #: --
      e-mail: info@climb.org.uk
      Home page: http://www.CLIMB.org.uk
    • Genetic and Rare Diseases (GARD) Information Center
      PO Box 8126
      Gaithersburg MD 20898-8126
      Phone #: 301-519-3194
      800 #: 888-205-2311
      e-mail: gardinfo@nih.gov
      Home page: http://www.genome.gov/10000409
    • MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
      150 Custer Court
      Green Bay WI 54301-1243
      Phone #: 920-336-5333
      800 #: 877-336-5333
      e-mail: mums@netnet.net
      Home page: http://www.netnet.net/mums/
    • Madisons Foundation
      PO Box 241956
      Los Angeles CA 90024
      Phone #: 310-264-0826
      800 #: N/A
      e-mail: getinfo@madisonsfoundation.org
      Home page: http://www.madisonsfoundation.org
    • Trimethylaminuria Foundation
      P.O. Box 3361
      New York NY 10163-3361
      Phone #: 212-300-4168
      800 #: --
      e-mail: theTFnetwk@aol.com
      Home page: N/A
    • Trimethylaminuria Midwest Region Foundation
      12537 Hardy Street
      Overland Park KS 66213-1446
      Phone #: 913-906-9496
      800 #: --
      e-mail: EMPORIA962000@YAHOO.COM
      Home page: N/A



    For a Complete Report

    This is an abstract of a report from the National Organization for Rare Disorders, Inc. ? (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html