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

    Gene ID: 2329, updated on 4-Jan-2025

    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_2024_08 current GRCh38.p14 (GCF_000001405.40) 1 NC_000001.11 (171314183..171342084)
    RS_2024_08 current T2T-CHM13v2.0 (GCF_009914755.1) 1 NC_060925.1 (170670481..170698383)
    RS_2024_09 previous assembly GRCh37.p13 (GCF_000001405.25) 1 NC_000001.10 (171283322..171311223)

    Chromosome 1 - NC_000001.11Genomic Context describing neighboring genes Neighboring gene FMO1 antisense RNA 1 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: Tissue-specific circular RNA induction during human fetal development
    • Description: 35 human fetal samples from 6 tissues (3 - 7 replicates per tissue) collected between 10 and 20 weeks gestational time were sequenced using Illumina TruSeq Stranded Total RNA
    • BioProject: PRJNA270632
    • Publication: PMID 26076956
    • Analysis date: Mon Apr 2 22:54:59 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)

    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 monooxygenase 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

      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_2024_08

    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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