Homo sapiens Protein: FMO3 | |||||||||||||||||||
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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.
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Gene Ontology | |||||||||||||||||||
Molecular Function |
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Biological Process |
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Cellular Component |
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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 |
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PFAM |
PF00743
PF07992 |
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PRINTS |
PR00370
PR01121 PR01122 PR01123 |
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PIRSF |
PIRSF000332
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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 | ||||||||||||||||||