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FMO4 flavin containing dimethylaniline monoxygenase 4 [ Homo sapiens (human) ]

Gene ID: 2329, updated on 5-Mar-2024

Summary

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Official Symbol
FMO4provided by HGNC
Official Full Name
flavin containing dimethylaniline monoxygenase 4provided by HGNC
Primary source
HGNC:HGNC:3772
See related
Ensembl:ENSG00000076258 MIM:136131; AllianceGenome:HGNC:3772
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
FMO2
Summary
Metabolic N-oxidation of diet-derived amino-trimethylamine (TMA) is mediated by flavin-containing monooxygenase and is subject to an inherited FMO3 polymorphism in man. This results in a small subpopulation with reduced TMA N-oxidation capacity and causes fish odor syndrome (Trimethylaminuria). Three forms of the enzyme are encoded by genes clustered in the 1q23-q25 region. Flavin-containing monooxygenases are NADPH-dependent flavoenzymes that catalyzes the oxidation of soft nucleophilic heteroatom centers in drugs, pesticides, and xenobiotics. [provided by RefSeq, Jan 2015]
Expression
Ubiquitous expression in kidney (RPKM 11.6), liver (RPKM 9.8) and 23 other tissues See more
Orthologs
mouse all
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Genomic context

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See FMO4 in Genome Data Viewer
Location:
1q24.3
Exon count:
11
Annotation release Status Assembly Chr Location
RS_2023_10 current GRCh38.p14 (GCF_000001405.40) 1 NC_000001.11 (171314183..171342084)
RS_2023_10 current T2T-CHM13v2.0 (GCF_009914755.1) 1 NC_060925.1 (170670481..170698383)
105.20220307 previous assembly GRCh37.p13 (GCF_000001405.25) 1 NC_000001.10 (171283322..171311223)

Chromosome 1 - NC_000001.11Genomic Context describing neighboring genes Neighboring gene uncharacterized LOC105371611 Neighboring gene uncharacterized LOC124900413 Neighboring gene flavin containing dimethylaniline monoxygenase 2 Neighboring gene NANOG-H3K27ac hESC enhancer GRCh37_chr1:171188772-171189333 Neighboring gene flavin containing dimethylaniline monoxygenase 1 Neighboring gene high mobility group box 1 pseudogene 11 Neighboring gene OCT4-NANOG-H3K27ac hESC enhancer GRCh37_chr1:171267702-171268494 Neighboring gene OCT4-NANOG-H3K27ac hESC enhancer GRCh37_chr1:171268495-171269285 Neighboring gene H3K27ac hESC enhancer GRCh37_chr1:171274060-171274560 Neighboring gene DNA topoisomerase I pseudogene 1 Neighboring gene SRP14 pseudogene 4 Neighboring gene GM2A pseudogene 2

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • 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 Apr 4 07:08:55 2018

Bibliography

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Related articles in PubMed

  1. Expression and characterization of a modified flavin-containing monooxygenase 4 from humans. Itagaki K, et al. J Biol Chem, 1996 Aug 16. PMID 8702731
  2. Cloning, primary sequence and chromosomal localization of human FMO2, a new member of the flavin-containing mono-oxygenase family. Dolphin CT, et al. Biochem J, 1992 Oct 1. PMID 1417778, Free PMC Article
  3. Quantification and cellular localization of expression in human skin of genes encoding flavin-containing monooxygenases and cytochromes P450. Janmohamed A, et al. Biochem Pharmacol, 2001 Sep 15. PMID 11551524
  4. The molecular biology of the flavin-containing monooxygenases of man. Phillips IR, et al. Chem Biol Interact, 1995 Apr 28. PMID 7720101
  5. Differential localization of flavin-containing monooxygenase (FMO) isoforms 1, 3, and 4 in rat liver and kidney and evidence for expression of FMO4 in mouse, rat, and human liver and kidney microsomes. Novick RM, et al. J Pharmacol Exp Ther, 2009 Jun. PMID 19307449, Free PMC Article

See all (16) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Observational study of gene-disease association. (HuGE Navigator)
    Title: Evaluation of candidate genes for cholinesterase activity in farmworkers exposed to organophosphorus pesticides: association of single nucleotide polymorphisms in BCHE.
  2. Data show a compelling visual demonstration of the isoform-specific localization patterns of FMO1, -3, and -4 in the rat liver and kidney and the first evidence for expression of FMO4 at the protein level in mouse and human liver and kidney microsomes.
    Title: Differential localization of flavin-containing monooxygenase (FMO) isoforms 1, 3, and 4 in rat liver and kidney and evidence for expression of FMO4 in mouse, rat, and human liver and kidney microsomes.
Submit: New GeneRIF Correction

Phenotypes

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Find tests for this gene in the NIH Genetic Testing Registry (GTR)

Review eQTL and phenotype association data in this region using PheGenI

Variation

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See variants in ClinVar

See studies and variants in dbVar

See Variation Viewer (GRCh37.p13)

See Variation Viewer (GRCh38)

Genotypes

Pathways from PubChem

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Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Markers

Homology

Gene Ontology Provided by GOA

Function Evidence Code Pubs
enables N,N-dimethylaniline monooxygenase activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables N,N-dimethylaniline monooxygenase activity NAS
Non-traceable Author Statement
more info
 PubMed 
enables NADP binding IEA
Inferred from Electronic Annotation
more info
  
enables flavin adenine dinucleotide binding IEA
Inferred from Electronic Annotation
more info
  
enables hypotaurine dehydrogenase activity IEA
Inferred from Electronic Annotation
more info
  
enables protein binding IPI
Inferred from Physical Interaction
more info
 PubMed 
Process Evidence Code Pubs
involved_in energy homeostasis IEA
Inferred from Electronic Annotation
more info
  
involved_in negative regulation of fatty acid oxidation IEA
Inferred from Electronic Annotation
more info
  
involved_in xenobiotic catabolic process IEA
Inferred from Electronic Annotation
more info
  
Component Evidence Code Pubs
located_in endoplasmic reticulum membrane IEA
Inferred from Electronic Annotation
more info
  

General protein information

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Preferred Names
dimethylaniline monooxygenase [N-oxide-forming] 4
Names
FMO 4
dimethylaniline oxidase 4
flavin containing monooxygenase 4
hepatic flavin-containing monooxygenase 4
NP_002013.1
XP_005245102.1
XP_005245103.1
XP_005245105.1
XP_006711306.1
XP_011507649.1
XP_011507650.1
XP_047272175.1
XP_047272184.1
XP_047272188.1
XP_054191466.1
XP_054191467.1
XP_054191468.1
XP_054191469.1
XP_054191470.1
XP_054191471.1
XP_054191472.1
XP_054191473.1
XP_054191474.1

NCBI Reference Sequences (RefSeq)

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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.

mRNA and Protein(s)

  1. NM_002022.3 → NP_002013.1  dimethylaniline monooxygenase [N-oxide-forming] 4

    See identical proteins and their annotated locations for NP_002013.1

    Status: REVIEWED

    Source sequence(s)
    BC002780, BX495448
    Consensus CDS
    CCDS1295.1
    UniProtKB/Swiss-Prot
    P31512, Q53XR0
    Related
    ENSP00000356723.3, ENST00000367749.4
    Conserved Domains (3) summary
    pfam00743
    Location:2 → 532
    FMO-like; Flavin-binding monooxygenase-like
    pfam13450
    Location:7 → 41
    NAD_binding_8; NAD(P)-binding Rossmann-like domain
    cl21454
    Location:89 → 220
    NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins

RefSeqs of Annotated Genomes: GCF_000001405.40-RS_2023_10

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

Reference GRCh38.p14 Primary Assembly

Genomic

  1. NC_000001.11 Reference GRCh38.p14 Primary Assembly

    Range
    171314183..171342084
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_006711243.4 → XP_006711306.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X6

    Conserved Domains (2) summary
    pfam00743
    Location:2 → 355
    FMO-like; Flavin-binding monooxygenase-like
    pfam13450
    Location:7 → 41
    NAD_binding_8; NAD(P)-binding Rossmann-like domain

  2. XM_005245048.4 → XP_005245105.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X7

    Conserved Domains (1) summary
    PLN02172
    Location:2 → 162
    PLN02172; flavin-containing monooxygenase FMO GS-OX

  3. XM_005245046.4 → XP_005245103.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X2

    Conserved Domains (3) summary
    pfam00743
    Location:2 → 469
    FMO-like; Flavin-binding monooxygenase-like
    pfam13450
    Location:7 → 41
    NAD_binding_8; NAD(P)-binding Rossmann-like domain
    cl21454
    Location:77 → 157
    NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins

  4. XM_005245045.4 → XP_005245102.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X1

    Conserved Domains (2) summary
    pfam00743
    Location:1 → 482
    FMO-like; Flavin-binding monooxygenase-like
    cl21454
    Location:39 → 170
    NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins

  5. XM_011509348.3 → XP_011507650.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X4

    Conserved Domains (1) summary
    pfam00743
    Location:1 → 389
    FMO-like; Flavin-binding monooxygenase-like

  6. XM_011509347.3 → XP_011507649.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X3

    Conserved Domains (3) summary
    pfam00743
    Location:2 → 436
    FMO-like; Flavin-binding monooxygenase-like
    pfam13450
    Location:7 → 41
    NAD_binding_8; NAD(P)-binding Rossmann-like domain
    cl21454
    Location:89 → 220
    NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins

  7. XM_047416228.1 → XP_047272184.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X8

  8. XM_047416219.1 → XP_047272175.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X5

  9. XM_047416232.1 → XP_047272188.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X9

Alternate T2T-CHM13v2.0

Genomic

  1. NC_060925.1 Alternate T2T-CHM13v2.0

    Range
    170670481..170698383
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_054335496.1 → XP_054191471.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X6

  2. XM_054335497.1 → XP_054191472.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X7

  3. XM_054335492.1 → XP_054191467.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X2

  4. XM_054335491.1 → XP_054191466.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X1

  5. XM_054335494.1 → XP_054191469.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X4

  6. XM_054335493.1 → XP_054191468.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X3

  7. XM_054335498.1 → XP_054191473.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X8

  8. XM_054335495.1 → XP_054191470.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X5

  9. XM_054335499.1 → XP_054191474.1  dimethylaniline monooxygenase [N-oxide-forming] 4 isoform X9

Nucleotide Protein
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
P31512.3 GenPept UniProtKB/Swiss-Prot:P31512

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