Trimethylaminuria

National Organization for Rare Disorders, Inc.

Important
It is possible that the main title of the report Trimethylaminuria is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

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

Disorder Subdivisions

  • None

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.

Resources

CLIMB (Children Living with Inherited Metabolic Diseases)
Climb Building
176 Nantwich Road
Crewe, Intl CW2 6BG
United Kingdom
Tel: +44 870 7700 325
Fax: +44 870 7700 327
Email: info.svcs@climb.org.uk
Internet: http://www.CLIMB.org.uk

Trimethylaminuria Foundation
P.O. Box 3361
Grand Central Station
New York, NY 10163-3361
USA
Tel: (212)300-4168
Fax: (917)640-7308
Email: theTFnetwk@aol.com

Trimethylaminuria Midwest Region Foundation
12537 Hardy Street
Overland Park, KS 66213-1446
Tel: (913)906-9496
Email: EMPORIA962000@YAHOO.COM

MUMS National Parent-to-Parent Network
150 Custer Court
Green Bay, WI 54301-1243
USA
Tel: (920)336-5333
Fax: (920)339-0995
Tel: (877)336-5333
Email: mums@netnet.net
Internet: http://www.netnet.net/mums/

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
TDD: (888)205-3223
Email: ordr@od.nih.gov
Internet: http://rarediseases.info.nih.gov/Default.aspx

Madisons Foundation
PO Box 241956
Los Angeles, CA 90024
Tel: (310)264-0826
Fax: (310)264-4766
Email: getinfo@madisonsfoundation.org
Internet: http://www.madisonsfoundation.org

For a Complete Report

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

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