Homo sapiens Protein: FMO3
Summary
InnateDB Protein IDBP-104770.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol FMO3
Protein Name flavin containing monooxygenase 3
Synonyms dJ127D3.1; FMOII; TMAU;
Species Homo sapiens
Ensembl Protein ENSP00000356729
InnateDB Gene IDBG-104768 (FMO3)
Protein Structure
UniProt Annotation
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 Localization Microsome membrane. Endoplasmic reticulum membrane.
Disease Associations Trimethylaminuria (TMAU) [MIM:602079]: 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. Affected 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. {ECO:0000269PubMed:10338091, ECO:0000269PubMed:10479479, ECO:0000269PubMed:11191884, ECO:0000269PubMed:12893987, ECO:0000269PubMed:9536088}. Note=The disease is caused by mutations affecting the gene represented in this entry.
Tissue Specificity Liver.
Comments
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 1 experimentally validated interaction(s) in this database.
Experimentally validated
Total 1 [view]
Protein-Protein 0
Protein-DNA 1 [view]
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Gene Ontology

Molecular Function
Accession GO Term
GO:0004499 N,N-dimethylaniline monooxygenase activity
GO:0016491 oxidoreductase activity
GO:0016597 amino acid binding
GO:0034899 trimethylamine monooxygenase activity
GO:0050660 flavin adenine dinucleotide binding
GO:0050661 NADP binding
Biological Process
GO:0006805 xenobiotic metabolic process
GO:0017144 drug metabolic process
GO:0044281 small molecule metabolic process
GO:0055114 oxidation-reduction process
Cellular Component
GO:0005789 endoplasmic reticulum membrane
GO:0016021 integral component of membrane
GO:0043231 intracellular membrane-bounded organelle
Protein Structure and Domains
PDB ID
InterPro IPR000960 Flavin monooxygenase FMO
IPR002253 Flavin monooxygenase (FMO) 1
IPR002254 Flavin monooxygenase (FMO) 2
IPR002255 Flavin monooxygenase (FMO) 3
IPR012143 Dimethylaniline monooxygenase, N-oxide-forming
IPR020946 Flavin monooxygenase-like
IPR023753 Pyridine nucleotide-disulphide oxidoreductase, FAD/NAD(P)-binding domain
PFAM PF00743
PF07992
PRINTS PR00370
PR01121
PR01122
PR01123
PIRSF PIRSF000332
SMART
TIGRFAMs
Post-translational Modifications
Modification
Cross-References
SwissProt P31513
PhosphoSite PhosphoSite-P31513
TrEMBL A0A024R8Z4
UniProt Splice Variant
Entrez Gene 2328
UniGene Hs.727057
RefSeq NP_008825
HUGO HGNC:3771
OMIM 136132
CCDS CCDS1292
HPRD 00633
IMGT
EMBL AK313197 AL021026 AY895830 BC032016 CH471067 M83772 U39961 U39962 U39963 U39964 U39965 U39966 U39967 Z47552
GenPept AAA86284 AAC51932 AAH32016 AAW65372 BAG36013 CAA15908 CAA87632 EAW90887 EAW90888