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    FMO1 flavin containing dimethylaniline monoxygenase 1 [ Homo sapiens (human) ]

    Gene ID: 2326, updated on 3-Dec-2024

    Summary

    Official Symbol
    FMO1provided by HGNC
    Official Full Name
    flavin containing dimethylaniline monoxygenase 1provided by HGNC
    Primary source
    HGNC:HGNC:3769
    See related
    Ensembl:ENSG00000010932 MIM:136130; AllianceGenome:HGNC:3769
    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
    Summary
    Metabolic N-oxidation of the diet-derived amino-trimethylamine (TMA) is mediated by flavin-containing monooxygenase and is subject to an inherited FMO3 polymorphism in man resulting in a small subpopulation with reduced TMA N-oxidation capacity resulting in fish odor syndrome Trimethylaminuria. Three forms of the enzyme, FMO1 found in fetal liver, FMO2 found in adult liver, and FMO3 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. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Sep 2013]
    Expression
    Biased expression in kidney (RPKM 54.7), small intestine (RPKM 8.2) and 1 other tissue See more
    Orthologs
    NEW
    Try the new Gene table
    Try the new Transcript table

    Genomic context

    See FMO1 in Genome Data Viewer
    Location:
    1q24.3
    Exon count:
    12
    Annotation release Status Assembly Chr Location
    RS_2024_08 current GRCh38.p14 (GCF_000001405.40) 1 NC_000001.11 (171248494..171285978)
    RS_2024_08 current T2T-CHM13v2.0 (GCF_009914755.1) 1 NC_060925.1 (170604793..170642278)
    RS_2024_09 previous assembly GRCh37.p13 (GCF_000001405.25) 1 NC_000001.10 (171217633..171255117)

    Chromosome 1 - NC_000001.11Genomic Context describing neighboring genes Neighboring gene uncharacterized LOC105371611 Neighboring gene flavin containing dimethylaniline monoxygenase 6, pseudogene Neighboring gene uncharacterized LOC124900413 Neighboring gene flavin containing dimethylaniline monoxygenase 2 Neighboring gene NANOG-H3K27ac hESC enhancer GRCh37_chr1:171188772-171189333 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 flavin containing dimethylaniline monoxygenase 4 Neighboring gene SRP14 pseudogene 4 Neighboring gene DNA topoisomerase I pseudogene 1

    Genomic regions, transcripts, and products

    Expression

    • 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

    GeneRIFs: Gene References Into Functions

    What's a GeneRIF?

    Phenotypes

    EBI GWAS Catalog

    Description
    GWAS for discovery and replication of genetic loci associated with sudden cardiac arrest in patients with coronary artery disease.
    EBI GWAS Catalog

    Pathways from PubChem

    Interactions

    Products Interactant Other Gene Complex Source Pubs Description

    General gene information

    Markers

    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 IDA
    Inferred from Direct Assay
    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 EXP
    Inferred from Experiment
    more info
    PubMed 
    enables hypotaurine monooxygenase activity IDA
    Inferred from Direct Assay
    more info
    PubMed 
    enables monooxygenase activity IDA
    Inferred from Direct Assay
    more info
    PubMed 
    enables protein binding IPI
    Inferred from Physical Interaction
    more info
    PubMed 
    enables trimethylamine monooxygenase activity ISS
    Inferred from Sequence or Structural Similarity
    more info
     
    Process Evidence Code Pubs
    involved_in NADPH oxidation IDA
    Inferred from Direct Assay
    more info
    PubMed 
    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 organic acid metabolic process IDA
    Inferred from Direct Assay
    more info
    PubMed 
    involved_in response to lipopolysaccharide IEA
    Inferred from Electronic Annotation
    more info
     
    involved_in sulfur amino acid catabolic process TAS
    Traceable Author Statement
    more info
     
    involved_in taurine biosynthetic process IDA
    Inferred from Direct Assay
    more info
    PubMed 
    involved_in toxin metabolic process IDA
    Inferred from Direct Assay
    more info
    PubMed 
    involved_in xenobiotic metabolic process IEA
    Inferred from Electronic Annotation
    more info
     
    Component Evidence Code Pubs
    located_in endoplasmic reticulum lumen TAS
    Traceable Author Statement
    more info
     
    located_in endoplasmic reticulum membrane ISS
    Inferred from Sequence or Structural Similarity
    more info
     

    General protein information

    Preferred Names
    flavin-containing monooxygenase 1
    Names
    FMO 1
    Flavin-containing monooxygenase 1 (fetal liver)
    dimethylaniline monooxygenase [N-oxide-forming] 1
    dimethylaniline oxidase 1
    fetal hepatic flavin-containing monooxygenase 1
    flavin containing monooxygenase 1
    trimethylamine monooxygenase
    NP_001269621.1
    NP_001269622.1
    NP_001269623.1
    NP_002012.1
    XP_005245094.1
    XP_005245095.1
    XP_006711304.1
    XP_006711305.1
    XP_054191390.1
    XP_054191391.1
    XP_054191392.1
    XP_054191393.1

    NCBI Reference Sequences (RefSeq)

    NEW Try the new Transcript table

    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_001282692.1NP_001269621.1  flavin-containing monooxygenase 1 isoform a

      Status: REVIEWED

      Description
      Transcript Variant: This variant (1) represents the longest transcript and encodes the longest isoform (a).
      Source sequence(s)
      BC047129, BE465459, DA750161
      UniProtKB/TrEMBL
      B2RCG5
      Related
      ENSP00000346901.4, ENST00000354841.4
      Conserved Domains (2) summary
      pfam00743
      Location:6536
      FMO-like; Flavin-binding monooxygenase-like
      cl21454
      Location:10214
      NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins
    2. NM_001282693.2NP_001269622.1  flavin-containing monooxygenase 1 isoform b

      See identical proteins and their annotated locations for NP_001269622.1

      Status: REVIEWED

      Description
      Transcript Variant: This variant (3) differs in the 5' UTR and coding sequence compared to variant 1. The resulting isoform (b) is shorter at the N-terminus compared to isoform a. Variants 2 and 3 both encode the same isoform (b).
      Source sequence(s)
      AK315100, DA537896, M64082
      Consensus CDS
      CCDS1294.1
      UniProtKB/Swiss-Prot
      A8K248, B7Z3P4, Q01740, Q5QPT2, Q9UJC2
      UniProtKB/TrEMBL
      B2RCG5
      Related
      ENSP00000481732.1, ENST00000617670.6
      Conserved Domains (1) summary
      pfam00743
      Location:2532
      FMO-like; Flavin-binding monooxygenase-like
    3. NM_001282694.2NP_001269623.1  flavin-containing monooxygenase 1 isoform c

      See identical proteins and their annotated locations for NP_001269623.1

      Status: REVIEWED

      Description
      Transcript Variant: This variant (4) differs in the 5' UTR and coding sequence and lacks an alternate in-frame exon compared to variant 1. The resulting isoform (c) is shorter at the N-terminus and lacks an alternate internal segment compared to isoform a.
      Source sequence(s)
      AK296198, DA537896, M64082
      Consensus CDS
      CCDS60351.1
      UniProtKB/TrEMBL
      B2RCG5
      Related
      ENSP00000385543.2, ENST00000402921.6
      Conserved Domains (2) summary
      pfam00743
      Location:2469
      FMO-like; Flavin-binding monooxygenase-like
      pfam13450
      Location:742
      NAD_binding_8; NAD(P)-binding Rossmann-like domain
    4. NM_002021.3NP_002012.1  flavin-containing monooxygenase 1 isoform b

      See identical proteins and their annotated locations for NP_002012.1

      Status: REVIEWED

      Description
      Transcript Variant: This variant (2) differs in the 5' UTR and coding sequence compared to variant 1. The resulting isoform (b) is shorter at the N-terminus compared to isoform a. Variants 2 and 3 both encode the same isoform (b).
      Source sequence(s)
      DA537896, M64082
      Consensus CDS
      CCDS1294.1
      UniProtKB/Swiss-Prot
      A8K248, B7Z3P4, Q01740, Q5QPT2, Q9UJC2
      UniProtKB/TrEMBL
      B2RCG5
      Related
      ENSP00000356724.3, ENST00000367750.7
      Conserved Domains (1) summary
      pfam00743
      Location:2532
      FMO-like; Flavin-binding monooxygenase-like

    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
      171248494..171285978
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_005245038.5XP_005245095.1  flavin-containing monooxygenase 1 isoform X4

      Conserved Domains (1) summary
      cl21454
      Location:6210
      NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins
    2. XM_006711241.5XP_006711304.1  flavin-containing monooxygenase 1 isoform X1

      UniProtKB/TrEMBL
      B2RCG5
      Conserved Domains (2) summary
      pfam00743
      Location:2497
      FMO-like; Flavin-binding monooxygenase-like
      cl21454
      Location:6210
      NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins
    3. XM_006711242.5XP_006711305.1  flavin-containing monooxygenase 1 isoform X3

      Conserved Domains (2) summary
      pfam00743
      Location:2418
      FMO-like; Flavin-binding monooxygenase-like
      cl21454
      Location:6210
      NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins
    4. XM_005245037.4XP_005245094.1  flavin-containing monooxygenase 1 isoform X2

      UniProtKB/TrEMBL
      B2RCG5
      Conserved Domains (2) summary
      pfam00743
      Location:9436
      FMO-like; Flavin-binding monooxygenase-like
      cl21454
      Location:4103
      NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins

    Alternate T2T-CHM13v2.0

    Genomic

    1. NC_060925.1 Alternate T2T-CHM13v2.0

      Range
      170604793..170642278
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_054335418.1XP_054191393.1  flavin-containing monooxygenase 1 isoform X4

    2. XM_054335415.1XP_054191390.1  flavin-containing monooxygenase 1 isoform X1

      UniProtKB/TrEMBL
      B2RCG5
    3. XM_054335417.1XP_054191392.1  flavin-containing monooxygenase 1 isoform X3

    4. XM_054335416.1XP_054191391.1  flavin-containing monooxygenase 1 isoform X2

      UniProtKB/TrEMBL
      B2RCG5