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

    Gene ID: 2326, updated on 2-Nov-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
    mouse all
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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: HPA RNA-seq normal tissues 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. 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)

    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 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

    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

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

    Gene LinkOut

    The following LinkOut resources are supplied by external providers. These providers are responsible for maintaining the links.

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