The Flavin containing monooxygenase family consists of five gene products, FMO1-5, that are major enzymatic oxidants involved in the metabolism of various therapeutics. Located in the liver, FMO3 is a hepatic microsomal enzyme that oxygenates soft nucleophiles such as secondary and tertiary amines. Through its N-oxygenase capabilities, FMO3 acts on a variety of xenobiotics to catalyze oxidative digestion. Defects in the FMO3 gene are the primary cause of trimethylaminuria (TMAuria), an inborn error of metabolism associated with a fishy body odor emitting from sweat, urine and breath. Genetic mutations in FMO3 lead to the N-oxidation of amino-trimethylamine derived from food products, thus producing the malodor associated with TMAuria.
Function:
Involved in the oxidative metabolism of a variety of xenobiotics such as drugs and pesticides. It N-oxygenates primary aliphatic alkylamines as well as secondary and tertiary amines. Plays an important role in the metabolism of trimethylamine (TMA), via the production of TMA N-oxide (TMAO). Is also able to perform S-oxidation when acting on sulfide compounds.
Subcellular Location:
Microsome membrane. Endoplasmic reticulum membrane.
Tissue Specificity:
Liver.
Post-translational modifications:
Belongs to the FMO family.
DISEASE:
Defects in FMO3 are the cause of trimethylaminuria (TMAU) [MIM:602079]; also known as fish-odor syndrome. TMAU is an inborn error of metabolism associated with an offensive body odor and caused by deficiency of FMO-mediated N-oxidation of amino-trimethylamine (TMA) derived from foodstuffs. Such individuals excrete relatively large amounts of TMA in their urine, sweat, and breath, and exhibit a fishy body odor characteristic of the malodorous free amine.
Similarity:
Belongs to the FMO family.
SWISS:
P31513
Gene ID:
2328
Database links:
Entrez Gene: 2328 Human
Entrez Gene: 14262 Mouse
Entrez Gene: 84493 Rat
Omim: 136132 Human
SwissProt: P31513 Human
SwissProt: P97501 Mouse
SwissProt: Q9EQ76 Rat
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