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Trimethyaminuria, also known as Fish Odour Syndrome, Fish Malodour Syndrome and Stale Fish Syndrome.
Trimethyaminuria is a metabolic disorder in which a person is not able to break down trimethylamine, the compound that gives fish their fishy odour, into smaller compounds.
This results in trimethylamine accumulating in the body and then being released in the person's sweat, urine and breath, giving off a strong fishy odour.
The intensity of this odour may vary over time. The strong body odour can interfere with many aspects of daily life, affecting a person's relationships, social life, and career. Some people with trimethylaminuria experience depression and social isolation as a result of this condition.
Although gene mutations account for most cases of trimethyaminuria, the condition can also be caused by other factors. A fish-like body odour may result from an excess of certain proteins in the diet or from an increase in bacteria that normally produce trimethylamine in the digestive system. A few cases of the disorder have been identified in adults with liver or kidney disease.
Trimethyaminuria is an uncommon genetic disorder. Mutations in the FMO3 gene cause trimethyaminuria.
The FMO3 gene provides instructions for making an enzyme that breaks down nitrogen-containing compounds from the diet, including trimethylamine. This compound is produced by bacteria in the intestine as they help digest proteins from eggs, liver, legumes (such as soybeans and peas), certain kinds of fish, and other foods. Normally, the FMO3 enzyme converts fishy-smelling trimethylamine into another molecule that has no odour. If the enzyme is missing or its activity is reduced because of a mutation in the FMO3 gene, trimethylamine is not processed properly and can build up in the body. As excess trimethylamine is released in a person's sweat, urine, and breath, it causes the strong odour characteristic of trimethyaminuria.
Most cases of trimethyaminuria appear to be inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. Most often, the parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but typically do not show signs and symptoms of the condition. Carriers of an FMO3 mutation, however, may have mild symptoms of trimethyaminuria or experience temporary episodes of fish-like body odour.
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