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Category Symbol Source: HGNC EntrezGene Ensembl GeneCards RNA genes CroW21

GIFtS
-

FMO3 Gene

protein-coding GIFtS: 66
GCID: GC01P171060

flavin containing monooxygenase 3

Explore 13 diseases affiliated with
FMO3 via

MalaCards

our new
Human Malady Compendium

(According to ¹HGNC, ²Entrez Gene,
³UniProtKB/Swiss-Prot, ⁴UniProtKB/TrEMBL, ⁵OMIM, ⁶GeneLoc, ⁷Ensembl, ⁸DME, ⁹miRBase, and/or ¹⁰fRNAdb)
About This Section

Aliases
Flavin Containing Monooxygenase 3¹ ²		EC 1.14.13.8³ ⁸
Dimethylaniline Oxidase 3² ³		TMAU² ⁵
Hepatic Flavin-Containing Monooxygenase 3² ³		FMOII²
Trimethylamine Monooxygenase² ³		DJ127D3.1¹
FMO 3² ³		Dimethylaniline Monooxygenase [N-Oxide-Forming] 3²
FMO Form 2² ³		Hepatic Flavin-Containing Monooxygenase-3²
FMO II² ³		EC 1.14.13.148³

External Ids:	HGNC: 3771¹	Entrez Gene: 2328²	Ensembl: ENSG00000007933⁷	OMIM: 136132⁵	UniProtKB: P31513³

Export aliases for FMO3 gene to outside databases

Previous GC identifers: GC01P168623 GC01P166689 GC0XP9N0049 GC01P168299 GC01P167791 GC01P169326 GC01P142283

(According to Entrez Gene, Tocris Bioscience, Wikipedia's Gene Wiki, PharmGKB,
UniProtKB/Swiss-Prot, and/or UniProtKB/TrEMBL)
About This Section

Entrez Gene summary for FMO3:

Flavin-containing monooxygenases (FMO) are an important class of drug-metabolizing enzymes that catalyze the

NADPH-dependent oxygenation of various nitrogen-,sulfur-, and phosphorous-containing xenobiotics such as therapeutic

drugs, dietary compounds, pesticides, and other foreign compounds. The human FMO gene family is composed of 5 genes

and multiple pseudogenes. FMO members have distinct developmental- and tissue-specific expression patterns. The

expression of this FMO3 gene, the major FMO expressed in adult liver, can vary up to 20-fold between individuals. This

inter-individual variation in FMO3 expression levels is likely to have significant effects on the rate at which

xenobiotics are metabolised and, therefore, is of considerable interest to the pharmaceutical industry. This

transmembrane protein localizes to the endoplasmic reticulum of many tissues. Alternative splicing of this gene

results in multiple transcript variants encoding the same protein. Mutations in this gene cause the disorder

trimethylaminuria (TMAu) which is characterized by the accumulation and excretion of unmetabolized trimethylamine and

a distinctive body odor. In healthy individuals, trimethylamine is primarily converted to the non odorous

trimethylamine N-oxide.(provided by RefSeq, Aug 2009)

UniProtKB/Swiss-Prot: FMO3_HUMAN, P31513

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

Gene Wiki entry for FMO3 (Flavin containing monooxygenase 3)

(According to GeneLoc and/or HGNC, and/or
Entrez Gene (NCBI build 37),
and/or miRBase,
Genomic Views according to UCSC (hg19) and Ensembl (release 69), Regulatory elements and Epigenetics data according to QIAGEN, SABiosciences, and/or SwitchGear Genomics)
About This Section

RefSeq DNA sequence:

NC_000001.10 NC_018912.1 NT_004487.19

Regulatory elements:

SABiosciences Regulatory transcription factor binding sites in the FMO3 gene promoter:
Max1 USF1 Pbx1a HNF-1A C/EBPalpha RORalpha2 Gfi-1 HNF-1 USF-1 c-Myc
Other transcription factors

SwitchGear Promoter luciferase reporter plasmid: FMO3 promoter sequence

Search SABiosciences Chromatin IP Primers for FMO3

Epigenetics:

QIAGEN PyroMark CpG Assay predesigned Pyrosequencing DNA Methylation assays in human, mouse, rat FMO3

Genomic Location:
Genomic View: UCSC Golden Path with GeneCards custom track

Entrez Gene cytogenetic band: 1q24.3 Ensembl cytogenetic band: 1q24.3 HGNC cytogenetic band: 1q24.3

FMO3 Gene in genomic location: bands according to Ensembl, locations according to (and/or Entrez Gene and/or Ensembl if different)
FMO3 gene location

GeneLoc information about chromosome 1 GeneLoc Exon Structure

GeneLoc location for GC01P171060: view genomic region (about GC identifiers)

Start:	171,060,018 bp from pter	End:	171,086,959 bp from pter
Size:	26,942 bases	Orientation:	plus strand

(According to ¹UniProtKB, HORDE, neXtProt, Ensembl, and/or Reactome, Modification sites according to ²PhosphoSitePlus, Specific Peptides from DME, Protein expression images according to data from SPIRE MOPED and PaxDb, RefSeq according to NCBI, PDB rendering according to OCA and/or Proteopedia, Recombinant Proteins from EMD Millipore, R&D Systems, GenScript, Enzo Life Sciences, OriGene, Novus Biologicals, Sino Biological, ProSpec, and/or Uscn,
Biochemical Assays by EMD Millipore, R&D Systems, OriGene, GenScript, Cell Signaling Technology, Enzo Life Sciences, and/or Uscn, Ontologies according to Gene Ontology Consortium 01 Mar 2013 and Entrez Gene, Antibodies by EMD Millipore, R&D Systems, GenScript, Cell Signaling Technology, OriGene, Novus Biologicals, Thermo Fisher Scientific, Abcam, and/or Uscn)
About This Section

UniProtKB/Swiss-Prot: FMO3_HUMAN, P31513 (See protein sequence)

Recommended Name: Dimethylaniline monooxygenase [N-oxide-forming] 3

Size: 532 amino acids; 60033 Da

Cofactor: FAD

Subcellular location: Microsome membrane. Endoplasmic reticulum membrane

Secondary accessions: B2R816 Q14854 Q8N5N5

Explore the universe of human proteins at neXtProt for FMO3: NX_P31513

Post-translational modifications:

View modification sites using PhosphoSitePlus²

View neXtProt modification sites for NX_P31513

4/23 DME Specific Peptides for FMO3 (P31513) (see all 23)

EFTETSA

FATGYSF

AVFDAVM

SKEMMCF

FMO3 Protein expression data from MOPED and PaxDb: About this image

REFSEQ proteins (2 alternative transcripts):

NP_001002294.1 NP_008825.4

ENSEMBL proteins:

ENSP00000356729 ENSP00000375935 ENSP00000439500 ENSP00000444073

Reactome Protein details: P31513
Human Recombinant Protein Products:

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	Novus Biologicals FMO3 Protein Novus Biologicals FMO3 Lysates
	Browse Sino Biological Recombinant Proteins
	Browse ProSpec Recombinant Proteins
	Uscn Proteins for FMO3

Gene Ontology (GO): 5 cellular component terms (GO ID links to tree view): About this table

GO ID	Qualified GO term	Evidence	PubMed IDs
GO:0005789	endoplasmic reticulum membrane	TAS	--
GO:0005792	microsome	--	--
GO:0016021	integral to membrane	IEA	--
GO:0031227	intrinsic to endoplasmic reticulum membrane	IEA	--
GO:0043231	intracellular membrane-bounded organelle	TAS	9207220

FMO3 for ontologies About GeneDecksing

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	Novus Biologicals FMO3 Antibodies
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Assay Products for FMO3:

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	Browse R&D Systems for biochemical assays
	GenScript Custom Assay Services for FMO3
	Browse Enzo Life Sciences for kits & assays
	Uscn ELISAs and CLIAs for FMO3

(According to InterPro, ProtoNet, UniProtKB, and/or BLOCKS, Sets of similar genes according to GeneDecks)
About This Section

FMO3 for domains About GeneDecksing

4 InterPro domains/families:

IPR020946 Flavin_mOase-like

IPR012143 DiMe-aniline_mOase

IPR002255 Flavin_mOase_3

IPR000960 Flavin_mOase

Graphical View of Domain Structure for InterPro Entry P31513

ProtoNet protein and cluster: P31513

3 Blocks protein families:

IPB000960 Flavin-containing monooxygenase (FMO) signature

IPB001327 Pyridine nucleotide-disulphide oxidoreductase

IPB002255 Flavin-containing monooxygenase (FMO) 3 signature

UniProtKB/Swiss-Prot: FMO3_HUMAN, P31513

Similarity: Belongs to the FMO family

(According to ¹UniProtKB, Genatlas, LifeMap Discovery™, IUBMB, and/or ²DME, Human phenotypes from GenomeRNAi, Animal models from MGI Mar 06 2013,
bound targets from SABiosciences, miRNA Gene Targets from miRTarBase shRNA from OriGene, RNAi from EMD Millipore, siRNAs from OriGene, QIAGEN, microRNA from QIAGEN, Gene Editing from DNA2.0, Clones from EMD Millipore, OriGene, SwitchGear Genomics, GenScript, Sino Biological, DNA2.0, and Vector BioLabs, Cell Lines from GenScript, LifeMap BioReagents, In Situ Hybridization Assays from Advanced Cell Diagnostics, Ontologies according to Gene Ontology Consortium 01 Mar 2013 via Entrez Gene.)
About This Section

Function Summary:

UniProtKB/Swiss-Prot: FMO3_HUMAN, P31513

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

Catalytic activity: N,N-dimethylaniline + NADPH + O(2) = N,N-dimethylaniline N-oxide + NADP(+) + H(2)O

Catalytic activity: N,N,N-trimethylamine + NADPH + O(2) = N,N,N-trimethylamine N-oxide + NADP(+) + H(2)O

Biophysicochemical properties: Kinetic parameters: KM=21 uM for trimethylamine (at pH 8.5); KM=31 uM for trimethylamine

(at pH 7.4 and 37 degrees Celsius); KM=43 uM for benzydamine (at pH 7.4 and 37 degrees Celsius); KM=55.7 uM for

ethylenethiourea (at pH 8.5); KM=71.8 uM for methimazole (at pH 8.5); KM=150.1 uM for sulindac (at pH 8.5); KM=248 uM

for methyl p-tolyl sulfide (at pH 7.4 and 37 degrees Celsius);

Genatlas biochemistry entry for FMO3:

flavin containing monooxygenase 3,microsomal,major hepatic form

Enzyme Numbers (IUBMB): EC 1.14.13.8¹ ² EC 1.14.13.148¹

miRNA Products:		OriGene 3'-UTR Clone (see all 2): FMO3 Browse MicroRNA Expression Plasmids
		QIAGEN Custom miScript Target Protector blocks miRNA-binding site of human, mouse, rat FMO3
		1 QIAGEN miScript miRNA Assays for microRNA that regulate FMO3:
		hsa-miR-3121-3p
		SwitchGear 3'UTR luciferase reporter plasmid: FMO3 3' UTR sequence

Inhib. RNA Products:		Browse for Gene Knock-down Tools from EMD Millipore
		OriGene 29mer shRNA kits in GFP-retroviral vector in human, mouse, rat for FMO3 (see all 7) OriGene shRNA RFP: FMO3 OriGene siRNA: FMO3
		QIAGEN FlexiTube/FlexiPlate siRNA for gene silencing in human, mouse, rat FMO3

Gene Editing
Products:

DNA2.0 Custom Protein Engineering Service for FMO3

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		GenScript: all cDNA clones in your preferred vector (see all 2): FMO3 (NM_001002294)
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Cell Line Products:		GenScript Custom overexpressing Cell Line Services for FMO3
		Search LifeMap BioReagents cell lines for FMO3

In Situ Assay
Products:

Advanced Cell Diagnostics RNAscope RNA in situ hybridization assays for FMO3

Gene Ontology (GO): 5 molecular function terms (GO ID links to tree view): About this table

GO ID	Qualified GO term	Evidence	PubMed IDs
GO:0004499	N,N-dimethylaniline monooxygenase activity	TAS	--
GO:0016597	amino acid binding	IEA	--
GO:0034899	trimethylamine monooxygenase activity	IEA	--
GO:0050660	flavin adenine dinucleotide binding	IEA	--
GO:0050661	NADP binding	IEA	--

FMO3 for ontologies About GeneDecksing

1 GenomeRNAi human phenotype for FMO3:

Decreased viability of wild-ty

(Pathways according to EMD Millipore, R&D Systems, Cell Signaling Technology, KEGG, PharmGKB, BioSystems, Reactome, Tocris Bioscience, GeneGo (Thomson Reuters), QIAGEN, and/or UniProtKB, Sets of similar genes according to GeneDecks, Interaction Networks according to SABiosciences, and/or STRING, Interactions according to ¹UniProtKB, ²MINT, ³I2D, and/or ⁴STRING, with links to IntAct and Ensembl, Ontologies according to Gene Ontology Consortium 01 Mar 2013 via Entrez Gene).
About This Section

Unified GeneCards pathways - 5/10 super-pathways (see all 10) About this table

See pathways by source

Super-pathway

contained gene-specific pathways

Nicotine Pathway, Pharmacokinetics

Nicotine Pathway, Pharmacokinetics

1.00

Nicotine metabolism

0.60

Catalytic cycle of mammalian FMOs

Catalytic cycle of mammalian FMOs

1.00

FMO oxidizes nucleophiles

0.60

Tamoxifen metabolism

Tamoxifen metabolism

1.00

Tamoxifen Pathway, Pharmacokinetics

0.68

Biological oxidations

Biological oxidations

1.00

metapathway biotransformation

0.41

Metabolism

Metabolism

1.00

Pathway sources
See GeneCards unified pathways
Show all pathways

5/6 BioSystems Pathways for FMO3 (see all 6)

	SIDS Susceptibility Pathways
	Catalytic cycle of mammalian FMOs
	Nicotine metabolism
	nicotine degradation IV
	Tamoxifen metabolism

4 Reactome Pathways for FMO3

	Metabolism
	Biological oxidations
	Phase 1 - Functionalization of compounds
	FMO oxidizes nucleophiles

3 PharmGKB Pathways for FMO3

	Busulfan Pathway, Pharmacodynamics
	Nicotine Pathway, Pharmacokinetics
	Tamoxifen Pathway, Pharmacokinetics

1 Kegg Pathway (Kegg details for FMO3):

Drug metabolism - cytochrome P450

FMO3 for pathways About GeneDecksing

Interactions:

Search SABiosciences Gene Network Central^TM Interacting Genes and Proteins Networks for FMO3

STRING Interaction Network Preview (showing 5 interactants - click image to see 10)

5/10 Interacting proteins for FMO3 (ENSP00000356729⁴) via UniProtKB, MINT, STRING, and/or I2D (see all 10)

Interactant		Interaction Details
GeneCard	External ID(s)	Interaction Details
CYP2D6	ENSP00000353820⁴	STRING: ENSP00000353820
CYP3A4	ENSP00000337915⁴	STRING: ENSP00000337915
CYP3A43	ENSP00000222382⁴	STRING: ENSP00000222382
CYP3A5	ENSP00000222982⁴	STRING: ENSP00000222982
CYP3A7	ENSP00000337450⁴	STRING: ENSP00000337450

About this table

Gene Ontology (GO): 3 biological process terms (GO ID links to tree view): About this table

GO ID	Qualified GO term	Evidence	PubMed IDs
GO:0006805	xenobiotic metabolic process	TAS	--
GO:0017144	drug metabolic process	IEA	--
GO:0044281	small molecule metabolic process	TAS	--

FMO3 for ontologies About GeneDecksing

(Chemical Compounds according to UniProtKB, Enzo Life Sciences, EMD Millipore, Tocris Bioscience HMDB, BitterDB, and/or Novoseek, and Drugs according to DrugBank, Enzo Life Sciences, and/or PharmGKB, with drugs/clinical trials/news search links to CenterWatch)
About This Section

FMO3 for compounds About GeneDecksing

Browse Small Molecules at EMD Millipore

Browse drugs & compounds from Enzo Life Sciences

Browse Tocris compounds for FMO3

9 HMDB Compounds for FMO3 About this table

Compound	Synonyms	CAS #	PubMed Ids
Dimethylaniline-N-oxide	Dimethylaniline N-oxide (see all 4)	874-52-2	--
FAD	1H-Purin-6-amine flavin dinucleotide (see all 21)	146-14-5	--
N,N-Dimethylaniline	Dimethylaminobenzene (see all 9)	121-69-7	--
NADP	Adenine-nicotinamide dinucleotide phosphate (see all 18)	53-59-8	--
NADPH	2'-(dihydrogen phosphate) 5'-(trihydrogen pyrophosphate) Adenosine 5'-ester with 1,4-dihydro-1-b-D-ribofuranosylnicotinamide (see all 23)	53-57-6	--
Oxygen	Oxygen (see all 5)	7782-44-7	--
Sulfide	Sulfide (see all 4)	18496-25-8	--
Trimethylamine	Dimethylmethaneamine (see all 6)	75-50-3	--
Water	Dihydrogen oxide (see all 2)	7732-18-5	--

8 DrugBank Compounds for FMO3 About this table

Compound	Synonyms	CAS #	Type	Actions	PubMed Ids
Cimetidine	--	51481-61-9	enzyme	substrate	19283698 11465082 7720103
Almotriptan	--	181183-52-8	enzyme	substrate	12642466
Clozapine	--	5786-21-0	enzyme	substrate	19515014
Dapsone	Acedapsone (see all 29)	80-08-0	enzyme	substrate	19515014
Dasatinib	--	302962-49-8	enzyme	substrate	18556438
Olanzapine	--	132539-06-1	enzyme	substrate	10511917
Tamoxifen	Tamoxifen Citrate (see all 5)	10540-29-1	enzyme	substrate	19515014
Voriconazole	--	137234-62-9	enzyme	substrate	19515014

10/30 Novoseek chemical compound relationships for FMO3 gene (see all 30) About this table

Compound	-log (P-Val)	Hits	PubMed IDs for Articles with Shared Sentences (# sentences)
methyl p-tolyl sulfide	90.2	6	17942081 (1), 12695352 (1), 17142560 (1), 19571433 (1)
benzydamine	85.7	9	11012553 (4), 15564885 (2), 17142560 (1), 19571433 (1)
sulindac sulfide	83.7	8	10807940 (1), 17559352 (1), 16214918 (1), 19321370 (1) (see all 5)
tmao	83	16	9776311 (2), 17531949 (1), 19881207 (1), 11465082 (1) (see all 9)
methimazole	81.7	7	12814961 (2), 11414682 (1), 9344459 (1), 17942081 (1)
thiobenzamide	80.4	1	15363661 (1)
s-(1,2-dichlorovinyl)-l-cysteine	73.1	2	17892265 (1), 9884308 (1)
n-octylamine	72.7	2	9750169 (1)
flavin	71.4	14	15623613 (1), 17302959 (1), 14709624 (1), 16214918 (1) (see all 12)
bufuralol	66.3	1	11038163 (1)

Search CenterWatch for drugs/clinical trials and news about FMO3

(Secondary structures according to fRNAdb,
GenBank/EMBL/DDBJ Accessions according to
Unigene (Build 235 Homo sapiens; Mar 10 2013) or GenBank,
RefSeq according to Entrez Gene,
DOTS (version 10), and/or AceView, transcript ids from Ensembl with links to UCSC,
exon structure from GeneLoc, alternative splicing isoforms according to ASD and/or ECgene,
RNAi Products from EMD Millipore,
siRNAs from OriGene, QIAGEN, shRNA from OriGene, microRNA from QIAGEN,
Tagged/untagged cDNA clones from OriGene, SwitchGear Genomics, GenScript, DNA2.0, Vector BioLabs, Primers from OriGene, SABiosciences, and/or QIAGEN )
About This Section

REFSEQ mRNAs for FMO3 gene (2 alternative transcripts):

NM_001002294.2 NM_006894.5

Unigene Cluster for FMO3:

Flavin containing monooxygenase 3

Hs.445350 [show with all ESTs]

Unigene Representative Sequence: AK223166

9 Ensembl transcripts including schematic representations, and UCSC links where relevant:

ENST00000367755(uc001ghi.3 uc001ghh.3 uc010pmb.2 uc010pmc.2)

ENST00000530212(uc010pma.1) ENST00000534514 ENST00000472784 ENST00000479749

ENST00000478457 ENST00000392085 ENST00000538429 ENST00000542847

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		1 QIAGEN miScript miRNA Assays for microRNA that regulate FMO3:
		hsa-miR-3121-3p
		SwitchGear 3'UTR luciferase reporter plasmid: FMO3 3' UTR sequence

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		QIAGEN QuantiTect SYBR Green Assays in human, mouse, rat FMO3
		QIAGEN QuantiFast Probe-based Assays in human, mouse, rat FMO3

Additional cDNA sequence:

AK223166.1 AK296105.1 AK298406.1 AK298456.1 AK310785.1 AK313197.1 BC032016.1 M83772.1

Z47552.1

12 DOTS entries:

DT.95166654 DT.413620 DT.92431685 DT.95166656 DT.92431687 DT.92431683 DT.99972987 DT.92042899

DT.92431692 DT.100767373 DT.92431689 DT.95166657

24/86 AceView cDNA sequences (see all 86):

BM989135 CA395462 AU107182 AV688561 AV648086 BU625130 H71848 AV654417

NM_006894 AU100084 AI052149 NM_001002294 AV699326 BC032016 T95250 BI758833

AI300119 AV648605 H37985 H38206 BM920762 AV648072 AV652829 AV697859

GeneLoc Exon Structure

4 Alternative Splicing Database (ASD) splice patterns (SP) for FMO3 About this scheme

ExUns:	1a	·	1b	·	1c	·	1d	^	2	^	3	^	4	^	5	^	6a	·	6b	^	7	^	8a	·	8b	^	9	^	10	^	11
SP1:											-		-												-
SP2:							-				-		-												-
SP3:							-
SP4:

ECgene alternative splicing isoforms for FMO3

(RNA expression data according to H-InvDB, NONCODE, miRBase, and RNAdb, Expression images according to data from BioGPS, Illumina Human BodyMap, and CGAP SAGE, Sets of similar genes according to GeneDecks, in vivo and in vitro expression data from LifeMap Discovery™, plus additional links to Genevestigator, and/or SOURCE, and/or BioGPS, and/or UniProtKB,
PCR Arrays from SABiosciences, Primers from OriGene, SABiosciences, and/or QIAGEN, In Situ Hybridization Assays from Advanced Cell Diagnostics)
About This Section

FMO3 expression in normal human tissues (normalized intensities)
See probesets specificity/sensitivity at GeneAnnot
About this imageBioGPS
CGAP TAG: ATTCCTTTTT

About this image

FMO3 expression in embryonic tissues and stem cells

Expression by the Database of Embryonic development, Stem cell research, and Regenerative medicine About this table

Stem Cell Differentiation: 4 LifeMap Cells

Name

Category

PureStem™ mesenchymal progenitor 7PEND24 (Embryonic Progenitor Cell)

Cartilage, Neural Crest

PureStem™ progenitor EN27 (Embryonic Progenitor Cell)

PureStem™ progenitor EN7 (Embryonic Progenitor Cell)

PureStem™ progenitor SM22 (Embryonic Progenitor Cell)

Expression:

Positive

Negative

Selective marker

Experimental details:

Curated

Microarrays

In-situ hybridization

See FMO3 Protein Expression from SPIRE MOPED and PaxDB
Genevestigator expression for FMO3

SOURCE GeneReport for Unigene cluster: Hs.445350

UniProtKB/Swiss-Prot: FMO3_HUMAN, P31513

Tissue specificity: Liver

SABiosciences Expression via Pathway-Focused PCR Arrays including FMO3:

Drug Metabolism: Phase I Enzymes in human mouse rat

Molecular Toxicology PathwayFinder 384HT in human mouse rat

Primer Products:		OriGene genome-wide validated SYBR primer pairs in human, mouse, rat for FMO3 Browse OriGene validated miRNA SYBR primer pairs
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		QIAGEN QuantiFast Probe-based Assays in human, mouse, rat FMO3

In Situ
Assay Products:

Advanced Cell Diagnostics RNAscope RNA in situ hybridization assays for FMO3

(Orthologs according to ^1,2HomoloGene (²older version, for species not in ¹newer version), ³euGenes, ⁴SGD , ⁵MGI Mar 06 2013, with possible further links to Flybase and/or WormBase, and/or ⁶Ensembl pan taxonomic compara , Gene Trees according to Ensembl and TreeFam)
About This Section

This gene was present in the common ancestor of eukaryotes.

Orthologs for FMO3 gene from 7/23 species (see all 23) About this table

Organism	Taxonomic classification	Gene	Description	Human Similarity	Orthology Type	Details
chicken (Gallus gallus)	Aves	Q8QH01_CHICK⁶	flavin containing monooxygenase 6	62(a)	1 → many	8(4952120-4960182)
lizard (Anolis carolinensis)	Reptilia	-- --	--	74(a) 70(a)	many ↔ many many ↔ many	AAWZ02037914(2376-9072) GL343300.1(1575635-1594346)
zebrafish (Danio rerio)	Actinopterygii	fmo5⁶ si:dkey-239i20.2⁶ (see all 5)	si:dkey-239i20.2 (see all 5)	50(a) 49(a) (see all 5)	many ↔ many many ↔ many (see all 5)	20(15099468-15110570) 20(15090312-15096721)
fruit fly (Drosophila melanogaster)	Insecta	Fmo-1⁶ Fmo-2⁶	Flavin-containing monooxygenase 2	29(a) 25(a)	possible ortholog possible ortholog	2R(19850498-19852164) 2R(2529923-2532600)
worm (Caenorhabditis elegans)	Secernentea	fmo-13³	DIMETHYLANILINE MONOOXYGENASE (N-OXIDE FORMING) more	42(a) (best of 6)		III(10766342-10770439) --
thale cress (Arabidopsis thaliana)	eudicotyledons	AT1G62620⁶ AT1G63370⁶ (see all 18)	flavin-containing monooxygenase-like protein (see all 18)	26(a) 26(a) (see all 18)	possible ortholog possible ortholog (see all 18)	1(23182644-23184991) 1(23503205-23505358)
rice (Oryza sativa)	Liliopsida	-- -- (see all 19)	flavin-containing monooxygenase family protein, pu... flavin-containing monooxygenase family protein, pu... (see all 19)	26(a) 25(a) (see all 19)	possible ortholog possible ortholog (see all 19)	7(676501-677907) Un(596161-597561)

ENSEMBL Gene Tree for FMO3 (if available)
TreeFam Gene Tree for FMO3 (if available)

(Paralogs according to ¹HomoloGene,
²Ensembl, and ³SIMAP, Pseudogenes according to ⁴Pseudogene.org Build 68)
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Paralogs for FMO3 gene

FMO2² FMO4² FMO5² FMO6P² FMO1²

6 SIMAP similar genes for FMO3 using alignment to 3 protein entries: FMO3_HUMAN (see all proteins):

FMO6P DKFZp667E137 FMO2 FMO1 FMO4 FMO5

FMO3 for paralogs About GeneDecksing

(SNPs/Variants according to the ¹NCBI SNP Database, ²Ensembl, ³PupaSUITE, UniProtKB, and DNA2.0, Linkage Disequilibrium by HapMap, Structural Variations(CNVs/InDels/Inversions) from the Database of Genomic Variants, Mutations from the Human Gene Mutation Database (HGMD) and the Locus Specific Mutation Databases (LSDB), Blood group antigen gene mutations by BGMUT, Resequencing Primers from QIAGEN, Cancer Mutation PCR Arrays and Assays and Copy Number PCR Arrays from SABiosciences)
About This Section

SNP ID	Valid	Clinical significance	Chr 1 pos	Sequence	#	AA Chg	Type	More	#	Allele freq	Pop	Total sample	More
Genomic Data					Transcription Related Data				Allele Frequencies
				--	--	--		--	--
rs2266782^1,2	C,F,O,H,	pathogenic	171076966(+)	`CAAAAG/AAGTCC`	4	/K /E	mis¹ ese³		58		NA EA NS MN WA CSA EU	13652
rs72549334^1,2	C	pathogenic	211900458(+)	`GGGACC/TGGTCG`	4	R W	mis¹		1		NA	4552
rs72549332^1,2	C	pathogenic	211900630(+)	`TATATA/GGATGA`	4	I M	mis¹		0	--	--	--	--
rs72549331^1,2	C,	pathogenic	211903436(+)	`GTCCCA/G/TCTGGG`	6	H R L	mis¹		1		NA	3560
rs28363581^1,2	C,F,H,	pathogenic	211903517(+)	`TCCTCT/CACTTG`	4	/P /L	mis¹ ese³ trp³		12		NS NA	5570
rs2266780^1,2	C,F,H,	pathogenic	211903673(+)	`CACAGA/GGACCT`	4	E G	mis¹ ese³		58		NA NS EA MN WA EU	12297
rs61753344^1,2	C,F,	pathogenic	211903683(+)	`TGAAGG/TAATTC`	4	E *	stg¹		2		NA EU	5873
rs1736557^1,2	C,F,O,A,H,	pathogenic	211907023(+)	`TGTACG/ATGAAG`	4	/M /V	mis¹ ese³		57		EA NA NS MN WA EU	13822
rs72549326^1,2	C,F	pathogenic	211910151(+)	`GTATCC/TCAACC`	4	P L	mis¹		2		NA EU	5867
rs72549325^1,2	C	pathogenic	211910167(+)	`GTTCCG/TGACAT`	4	G *	stg¹		0	--	--	--	--

HapMap Linkage Disequilibrium report for FMO3 (171060018 - 171086959 bp)
Structural Variations (Copy Number Variations, Insertions/Deletions, Inversions)
Database of Genomic Variants (DGV): 1 variation for FMO3
1 CNV: 70415
Human Gene Mutation Database (HGMD): FMO3

Locus Specific Mutation Databases (LSDB): FMO3

SABiosciences Cancer Mutation PCR Assays

QIAGEN SeqTarget long-range PCR primers in human, mouse, rat for resequencing FMO3

DNA2.0 Custom Variant and Variant Library Synthesis for FMO3

(in which this Gene is Involved, According to MalaCards, OMIM, UniProtKB, the University of Copenhagen DISEASES database, Novoseek, Genatlas, GeneTests, GAD, HuGE Navigator, and/or TGDB.)
About This Section

FMO3 for disorders           About GeneDecksing

OMIM gene information: 136132
OMIM disorders: 602079
UniProtKB/Swiss-Prot: FMO3_HUMAN, P31513

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

13 diseases for FMO3: About MalaCards

trimethylaminuria sudden infant death syndrome familial adenomatous polyposis patent ductus arteriosus

hepatitis metabolic disorders polyposis hearing loss

essential hypertension adenoma hypertension colorectal cancer

schizophrenia

5 Novoseek disease relationships for FMO3 gene About this table

Disease	-log (P-Val)	Hits	PubMed IDs for Articles with Shared Sentences (# sentences)
trimethylaminuria	98.2	85	17584019 (4), 9282831 (4), 19881207 (3), 16601883 (3) (see all 31)
metabolic disorder	40.6	6	17584019 (1), 17531949 (1), 17142560 (1)
familial adenomatous polyposis	27.1	2	17559352 (1)
polyposis	25.4	3	15623613 (2), 16214918 (1)
adenoma	0	1	16214918 (1)

GeneTests: FMO3

Trimethylaminuria

Genetic Association Database (GAD): FMO3
Human Genome Epidemiology (HuGE) Navigator: FMO3 (23 documents)

Export disorders for FMO3 gene to outside databases

(in PubMed. Associations of this gene to articles via ¹Entrez Gene, ²UniProtKB/Swiss-Prot, ³HGNC, ⁴GAD, ⁵PharmGKB, ⁶HMDB, ⁷DrugBank, ⁸UniProtKB/TrEMBL, ⁹ Novoseek, and/or ¹⁰fRNAdb)
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PubMed articles for FMO3 gene, integrated from 9 sources (see all 162):
(articles sorted by number of sources associating them with FMO3)

Utopia: connect your pdf to the dynamic
world of online information

Structural organization of the human flavin-containing monooxygenase 3 gene (FMO3), the favored candidate for fish-odor syndrome, determined directly from genomic DNA. (PubMed id 9417913)^{1, 2, 3, 9} Dolphin C.T.... Phillips I.R. (1997)
Two new polymorphisms of the FMO3 gene in Caucasian and African-American populations: comparative genetic and functional studies. (PubMed id 12814961)^{1, 4, 9} Lattard V....Cashman J.R. (2003)
Population distribution of human flavin-containing monooxygenase form 3: gene polymorphisms. (PubMed id 11717182)^{1, 4, 9} Cashman J.R....Braun A. (2001)
Compound heterozygosity for missense mutations in the flavin- containing monooxygenase 3 (FM03) gene in patients with fish-odour syndrome. (PubMed id 11191884)^{1, 2, 9} Dolphin C.T.... Phillips I.R. (2000)
Two novel single nucleotide polymorphisms (SNPs) of the FMO3 gene in Japanese. (PubMed id 15618753)^{1, 2, 9} Fujieda M.... Kamataki T. (2003)
Polymorphisms of the Flavin containing monooxygenase 3 (FMO3) gene do not predispose to essential hypertension in Caucasians. (PubMed id 16324215)^{1, 4, 9} Dolan C....Treacy E.P. (2005)
[The frequency distribution of flavin-containing monooxygenase 3 mutant alleles in 28 populations from Yunnan] (PubMed id 12903042)^{1, 4, 9} Zeng W.M....Xiao C.J. (2003)
Functional characterization of genetic variants of human FMO3 associated with trimethylaminuria. (PubMed id 17531949)^{1, 2, 9} Yeung C.K....Rettie A.E. (2007)
Genetic polymorphisms of flavin monooxygenase 3 in sulindac-induced regression of colorectal adenomas in familial adenomatous polyposis. (PubMed id 16214918)^{1, 4, 9} Hisamuddin I.M....Yang V.W. (2005)
Trimethylaminuria is caused by mutations of the FMO3 gene in a North American cohort. (PubMed id 10479479)^{1, 2, 9} Akerman B.R.... Treacy E.P. (1999)

(in PubMed, OMIM, and NCBI Bookshelf)
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		AND	OR
Aliases
Disorders
Free Text

Query String		PubMed OMIM NCBI Bookshelf
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(According to Entrez Gene, HGNC, AceView, euGenes, Ensembl, miRBase, ECgene, Kegg, and/or H-InvDB)
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Entrez Gene: 2328	HGNC: 3771	AceView: FMO3	Ensembl:ENSG00000007933	euGenes: HUgn2328
ECgene: FMO3	Kegg: 2328	H-InvDB: FMO3

(According to HUGE)
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(According to PharmGKB, ATLAS, HORDE, IMGT, LEIDEN, UniProtKB/Swiss-Prot, and/or UniProtKB/TrEMBL,
Wikipedia and/or GeneReviews via UniProtKB/Swiss-Prot)
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Name	Description
PharmGKB entry for FMO3	Pharmacogenomics, SNPs, Pathways
GeneReviews	http://www.ncbi.nlm.nih.gov/sites/GeneTests/lab/gene/FMO3
NIEHS-SNPs	http://egp.gs.washington.edu/data/fmo3/

Intellectual Property for FMO3 gene
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Patent Information for FMO3 gene:
Search GeneIP for patents involving FMO3

GeneCards and IP:
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flavin containing monooxygenase 3

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FMO3 Gene

flavin containing monooxygenase 3

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