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

Gene ID: 2326, updated on 10-Dec-2024

Summary

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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
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Genomic context

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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

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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. Increased incidence of FMO1 gene single nucleotide polymorphisms in sporadic amyotrophic lateral sclerosis. Cereda C, et al. Amyotroph Lateral Scler, 2006 Dec. PMID 17127561
  2. Cloning, primary sequence, and chromosomal mapping of a human flavin-containing monooxygenase (FMO1). Dolphin C, et al. J Biol Chem, 1991 Jul 5. PMID 1712018
  3. Immunoquantitation of FMO1 in human liver, kidney, and intestine. Yeung CK, et al. Drug Metab Dispos, 2000 Sep. PMID 10950857
  4. Identification of human drug-metabolizing enzymes involved in the metabolism of SNI-2011. Washio T, et al. Biol Pharm Bull, 2001 Nov. PMID 11725960
  5. Cytochrome P450 2C8 and flavin-containing monooxygenases are involved in the metabolism of tazarotenic acid in humans. Attar M, et al. Drug Metab Dispos, 2003 Apr. PMID 12642475

See all (47) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Human flavin-containing monooxygenase 1 and its long-sought hydroperoxyflavin intermediate.
    Title: Human flavin-containing monooxygenase 1 and its long-sought hydroperoxyflavin intermediate.
  2. Decreases in N'-oxidation activity (V (max)/K (m)) were observed for the FMO1(I303V), FMO3(N61S), FMO3(D132H), FMO3(V257M), and FMO3(E308G) variants in vitro when compared with their respective wild-type isoforms.
    Title: Nicotine-N'-Oxidation by Flavin Monooxygenase Enzymes.
  3. Differential gene expression by SFRP2(+), FMO1(+), and COL11A1(+) fibroblasts suggests roles in matrix deposition, inflammatory cell retention, and connective tissue cell differentiation, respectively.
    Title: SFRP2/DPP4 and FMO1/LSP1 Define Major Fibroblast Populations in Human Skin.
  4. Report developmental regulation of hepatic FMO1 expression.
    Title: Quantification of Flavin-containing Monooxygenases 1, 3, and 5 in Human Liver Microsomes by UPLC-MRM-Based Targeted Quantitative Proteomics and Its Application to the Study of Ontogeny.
  5. Study tested the genetic effects of three FMOs genes (FMO1, FMO3, and FMO6P) on nicotine dependence by performing targeted sequencing on 2,852 nicotine-dependent and nondependent smokers; identified significant association signals for gene FMO1 and FMO6P
    Title: Targeted sequencing identifies genetic polymorphisms of flavin-containing monooxygenase genes contributing to susceptibility of nicotine dependence in European American and African American.
  6. data support the role of FMO3 in the N-oxidation of OLA and implicate for the first time the contribution of FMO1 and its functional *6 variant in OLA disposition
    Title: Influence of FMO1 and 3 polymorphisms on serum olanzapine and its N-oxide metabolite in psychiatric patients.
  7. polymorphisms in FMO1 are significant risk factors in the development of nicotine dependence and that the mechanism may involve variation in nicotine pharmacology.
    Title: Common polymorphisms in FMO1 are associated with nicotine dependence.
  8. The results of this study suggested that the alteration of FMO gene expression is a consequence of the pathological environment linked to oxidative stress related to mutated SOD1.
    Title: Flavin-containing monooxygenase mRNA levels are up-regulated in als brain areas in SOD1-mutant mice.
  9. Observational study of gene-disease association and gene-environment interaction. (HuGE Navigator)
    Title: Childhood brain tumors, residential insecticide exposure, and pesticide metabolism genes.
  10. The resulting data showed that N,N-dimethylamphetamine N-oxidation is mainly mediated by FMO1.
    Title: Flavin-containing monooxygenase 1-catalysed N,N-dimethylamphetamine N-oxidation.
Submit: New GeneRIF Correction See all GeneRIFs (17)

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

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

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 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

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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)

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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_001282692.1 → NP_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:6 → 536
    FMO-like; Flavin-binding monooxygenase-like
    cl21454
    Location:10 → 214
    NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins

  2. NM_001282693.2 → NP_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:2 → 532
    FMO-like; Flavin-binding monooxygenase-like

  3. NM_001282694.2 → NP_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:2 → 469
    FMO-like; Flavin-binding monooxygenase-like
    pfam13450
    Location:7 → 42
    NAD_binding_8; NAD(P)-binding Rossmann-like domain

  4. NM_002021.3 → NP_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:2 → 532
    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_005245038.5 → XP_005245095.1  flavin-containing monooxygenase 1 isoform X4

    Conserved Domains (1) summary
    cl21454
    Location:6 → 210
    NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins

  2. XM_006711241.5 → XP_006711304.1  flavin-containing monooxygenase 1 isoform X1

    UniProtKB/TrEMBL
    B2RCG5
    Conserved Domains (2) summary
    pfam00743
    Location:2 → 497
    FMO-like; Flavin-binding monooxygenase-like
    cl21454
    Location:6 → 210
    NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins

  3. XM_006711242.5 → XP_006711305.1  flavin-containing monooxygenase 1 isoform X3

    Conserved Domains (2) summary
    pfam00743
    Location:2 → 418
    FMO-like; Flavin-binding monooxygenase-like
    cl21454
    Location:6 → 210
    NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins

  4. XM_005245037.4 → XP_005245094.1  flavin-containing monooxygenase 1 isoform X2

    UniProtKB/TrEMBL
    B2RCG5
    Conserved Domains (2) summary
    pfam00743
    Location:9 → 436
    FMO-like; Flavin-binding monooxygenase-like
    cl21454
    Location:4 → 103
    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_054335418.1 → XP_054191393.1  flavin-containing monooxygenase 1 isoform X4

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

    UniProtKB/TrEMBL
    B2RCG5

  3. XM_054335417.1 → XP_054191392.1  flavin-containing monooxygenase 1 isoform X3

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

    UniProtKB/TrEMBL
    B2RCG5
Nucleotide Protein
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q01740.3 GenPept UniProtKB/Swiss-Prot:Q01740

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