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

Gene ID: 2326, updated on 7-Jun-2020

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
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

Genomic context

See FMO1 in Genome Data Viewer
Location:
1q24.3
Exon count:
11
Annotation release Status Assembly Chr Location
109.20200522 current GRCh38.p13 (GCF_000001405.39) 1 NC_000001.11 (171248471..171285978)
105 previous assembly GRCh37.p13 (GCF_000001405.25) 1 NC_000001.10 (171217610..171255113)

Chromosome 1 - NC_000001.11Genomic Context describing neighboring genes Neighboring gene uncharacterized LOC105371611 Neighboring gene flavin containing dimethylaniline monoxygenase 6, pseudogene Neighboring gene flavin containing dimethylaniline monoxygenase 2 Neighboring gene high mobility group box 1 pseudogene 11 Neighboring gene flavin containing dimethylaniline monoxygenase 4 Neighboring gene signal recognition particle 14 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

NHGRI GWAS Catalog

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

Pathways from PubChem

Interactions

Products Interactant Other Gene Complex Source Pubs Description

General gene information

Markers

Homology

Gene Ontology Provided by GOA

Function Evidence Code Pubs
N,N-dimethylaniline monooxygenase activity IDA
Inferred from Direct Assay
more info
PubMed 
NADP binding IEA
Inferred from Electronic Annotation
more info
 
flavin adenine dinucleotide binding IEA
Inferred from Electronic Annotation
more info
 
monooxygenase activity IBA
Inferred from Biological aspect of Ancestor
more info
PubMed 
monooxygenase activity IDA
Inferred from Direct Assay
more info
PubMed 
protein binding IPI
Inferred from Physical Interaction
more info
PubMed 
Process Evidence Code Pubs
NADPH oxidation IDA
Inferred from Direct Assay
more info
PubMed 
drug metabolic process IEA
Inferred from Electronic Annotation
more info
 
organic acid metabolic process IDA
Inferred from Direct Assay
more info
PubMed 
response to lipopolysaccharide IEA
Inferred from Electronic Annotation
more info
 
response to osmotic stress IEA
Inferred from Electronic Annotation
more info
 
toxin metabolic process IDA
Inferred from Direct Assay
more info
PubMed 
xenobiotic metabolic process TAS
Traceable Author Statement
more info
 
Component Evidence Code Pubs
endoplasmic reticulum lumen TAS
Traceable Author Statement
more info
 
endoplasmic reticulum membrane IEA
Inferred from Electronic Annotation
more info
 
integral component of membrane IEA
Inferred from Electronic Annotation
more info
 

General protein information

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

NCBI Reference Sequences (RefSeq)

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  dimethylaniline monooxygenase [N-oxide-forming] 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/Swiss-Prot
    Q01740
    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  dimethylaniline monooxygenase [N-oxide-forming] 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
    Q01740
    UniProtKB/TrEMBL
    A0A024R934, B2RCG5
    Related
    ENSP00000481732.1, ENST00000617670.6
    Conserved Domains (1) summary
    pfam00743
    Location:2532
    FMO-like; Flavin-binding monooxygenase-like
  3. NM_001282694.1NP_001269623.1  dimethylaniline monooxygenase [N-oxide-forming] 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, BE465459, DA537896, M64082
    Consensus CDS
    CCDS60351.1
    UniProtKB/Swiss-Prot
    Q01740
    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  dimethylaniline monooxygenase [N-oxide-forming] 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
    Q01740
    UniProtKB/TrEMBL
    A0A024R934
    Related
    ENSP00000356724.3, ENST00000367750.7
    Conserved Domains (1) summary
    pfam00743
    Location:2532
    FMO-like; Flavin-binding monooxygenase-like

RefSeqs of Annotated Genomes: Homo sapiens Updated Annotation Release 109.

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

Reference GRCh38.p13 Primary Assembly

Genomic

  1. NC_000001.11 Reference GRCh38.p13 Primary Assembly

    Range
    171248471..171285978
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_005245038.4XP_005245095.1  dimethylaniline monooxygenase [N-oxide-forming] 1 isoform X4

    Conserved Domains (1) summary
    cl21454
    Location:6210
    NADB_Rossmann; Rossmann-fold NAD(P)(+)-binding proteins
  2. XM_006711241.4XP_006711304.1  dimethylaniline monooxygenase [N-oxide-forming] 1 isoform X1

    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_006711242.4XP_006711305.1  dimethylaniline monooxygenase [N-oxide-forming] 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_005245037.3XP_005245094.1  dimethylaniline monooxygenase [N-oxide-forming] 1 isoform X2

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