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FMO3 flavin containing monooxygenase 3 [ Homo sapiens (human) ]

Gene ID: 2328, updated on 3-Jun-2018
Official Symbol
FMO3provided by HGNC
Official Full Name
flavin containing monooxygenase 3provided by HGNC
Primary source
HGNC:HGNC:3771
See related
Ensembl:ENSG00000007933 MIM:136132; Vega:OTTHUMG00000035505
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Homo sapiens
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as
TMAU; FMOII; dJ127D3.1
Summary
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 different isoforms. 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, Jan 2016]
Expression
Biased expression in liver (RPKM 241.9) and adrenal (RPKM 8.6) See more
Orthologs
See FMO3 in Genome Data Viewer
Location:
1q24.3
Exon count:
10
Annotation release Status Assembly Chr Location
109 current GRCh38.p12 (GCF_000001405.38) 1 NC_000001.11 (171090873..171117819)
105 previous assembly GRCh37.p13 (GCF_000001405.25) 1 NC_000001.10 (171060018..171086961)

Chromosome 1 - NC_000001.11Genomic Context describing neighboring genes Neighboring gene maestro heat like repeat family member 9 Neighboring gene alkylated DNA repair protein alkB homolog 8-like Neighboring gene uncharacterized LOC105371611 Neighboring gene microRNA 1295a Neighboring gene microRNA 1295b Neighboring gene flavin containing monooxygenase 6 pseudogene Neighboring gene flavin containing monooxygenase 2

  • Project title: HPA RNA-seq normal tissues
  • Description: RNA-seq was performed of tissue samples from 95 human individuals representing 27 different tissues in order to determine tissue-specificity of all protein-coding genes
  • BioProject: PRJEB4337
  • Publication: PMID 24309898
  • Analysis date: Wed Jun 15 11:32:44 2016

GeneRIFs: Gene References Into FunctionsWhat's a GeneRIF?

Associated conditions

Description Tests
Trimethylaminuria
MedGen: C0342739 OMIM: 602079 GeneReviews: Primary Trimethylaminuria
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NHGRI GWAS Catalog

Description
An atlas of genetic influences on human blood metabolites.
NHGRI GWA Catalog
Genome-wide association identifies genetic variants associated with lentiform nucleus volume in N = 1345 young and elderly subjects.
NHGRI GWA Catalog

Protein interactions

Protein Gene Interaction Pubs
Tat tat HIV-1 Tat upregulates flavin containing monooxygenase 3 (FMO3) in HEK 293T cells PubMed

Go to the HIV-1, Human Interaction Database

Markers

Homology

Clone Names

  • MGC34400

Gene Ontology Provided by GOA

Function Evidence Code Pubs
N,N-dimethylaniline monooxygenase activity TAS
Traceable Author Statement
more info
 
NADP binding IEA
Inferred from Electronic Annotation
more info
 
flavin adenine dinucleotide binding IEA
Inferred from Electronic Annotation
more info
 
monooxygenase activity IBA
Inferred from Biological aspect of Ancestor
more info
PubMed 
protein binding IPI
Inferred from Physical Interaction
more info
PubMed 
trimethylamine monooxygenase activity IEA
Inferred from Electronic Annotation
more info
 
Process Evidence Code Pubs
xenobiotic metabolic process TAS
Traceable Author Statement
more info
 
Component Evidence Code Pubs
endoplasmic reticulum membrane TAS
Traceable Author Statement
more info
 
integral component of membrane IEA
Inferred from Electronic Annotation
more info
 
intracellular membrane-bounded organelle TAS
Traceable Author Statement
more info
PubMed 
organelle membrane IEA
Inferred from Electronic Annotation
more info
 
Preferred Names
dimethylaniline monooxygenase [N-oxide-forming] 3
Names
FMO form 2
dimethylaniline oxidase 3
hepatic flavin-containing monooxygenase-3
trimethylamine monooxygenase
NP_001002294.1
NP_001306102.1
NP_001306103.1
NP_008825.4
XP_011507647.1
XP_024310133.1

RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

Genomic

  1. NG_012690.1 RefSeqGene

    Range
    5001..31942
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_001002294.2NP_001002294.1  dimethylaniline monooxygenase [N-oxide-forming] 3 isoform a

    See identical proteins and their annotated locations for NP_001002294.1

    Status: REVIEWED

    Description
    Transcript Variant: This variant (2) encodes the longest isoform (a). Variants 1 and 2 both encode the same isoform (a).
    Source sequence(s)
    AK223166, AK313197, BC032016, BM453951
    Consensus CDS
    CCDS1292.1
    UniProtKB/Swiss-Prot
    P31513
    UniProtKB/TrEMBL
    A0A024R8Z4, Q53FW5
    Related
    ENSP00000356729.4, OTTHUMP00000033540, ENST00000367755.8, OTTHUMT00000086219
    Conserved Domains (2) summary
    pfam00743
    Location:2532
    FMO-like; Flavin-binding monooxygenase-like
    pfam13450
    Location:741
    NAD_binding_8; NAD(P)-binding Rossmann-like domain
  2. NM_001319173.1NP_001306102.1  dimethylaniline monooxygenase [N-oxide-forming] 3 isoform b

    Status: REVIEWED

    Description
    Transcript Variant: This variant (3) uses an alternate 5' splice junction and contains an alternate exon compared to variant 2. The resulting isoform (b) has a shorter and distinct N-terminus compared to isoform a.
    Source sequence(s)
    AK223166, AK298406, AK313197, BC032016, BM453951
    UniProtKB/Swiss-Prot
    P31513
    UniProtKB/TrEMBL
    B7Z543, Q53FW5
    Conserved Domains (1) summary
    pfam00743
    Location:25512
    FMO-like; Flavin-binding monooxygenase-like
  3. NM_001319174.1NP_001306103.1  dimethylaniline monooxygenase [N-oxide-forming] 3 isoform c

    Status: REVIEWED

    Description
    Transcript Variant: This variant (4) lacks an alternate in-frame exon compared to variant 2. The resulting isoform (c) has the same N- and C-termini but is shorter compared to isoform a.
    Source sequence(s)
    AK223166, AK296105, AK313197, AL021026, BC032016, BM453951
    UniProtKB/Swiss-Prot
    P31513
    UniProtKB/TrEMBL
    B7Z3M2, Q53FW5
    Conserved Domains (2) summary
    pfam00743
    Location:2469
    FMO-like; Flavin-binding monooxygenase-like
    pfam13450
    Location:741
    NAD_binding_8; NAD(P)-binding Rossmann-like domain
  4. NM_006894.5NP_008825.4  dimethylaniline monooxygenase [N-oxide-forming] 3 isoform a

    See identical proteins and their annotated locations for NP_008825.4

    Status: REVIEWED

    Description
    Transcript Variant: This variant (1) differs in the 5' UTR compared to variant 2. Variants 1 and 2 both encode the same isoform (a).
    Source sequence(s)
    AK223166, AK313197, BC032016, BM453951
    Consensus CDS
    CCDS1292.1
    UniProtKB/Swiss-Prot
    P31513
    UniProtKB/TrEMBL
    A0A024R8Z4, Q53FW5
    Conserved Domains (2) summary
    pfam00743
    Location:2532
    FMO-like; Flavin-binding monooxygenase-like
    pfam13450
    Location:741
    NAD_binding_8; NAD(P)-binding Rossmann-like domain

RefSeqs of Annotated Genomes: Homo sapiens Annotation Release 109 details...Open this link in a new tab

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCh38.p12 Primary Assembly

Genomic

  1. NC_000001.11 Reference GRCh38.p12 Primary Assembly

    Range
    171090873..171117819
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_011509345.3XP_011507647.1  dimethylaniline monooxygenase [N-oxide-forming] 3 isoform X1

    See identical proteins and their annotated locations for XP_011507647.1

    Conserved Domains (1) summary
    pfam00743
    Location:25512
    FMO-like; Flavin-binding monooxygenase-like
  2. XM_024454365.1XP_024310133.1  dimethylaniline monooxygenase [N-oxide-forming] 3 isoform X2

    Conserved Domains (1) summary
    cl26174
    Location:25283
    K_oxygenase; L-lysine 6-monooxygenase (NADPH-requiring)
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