1. Gene
  2. FMO3 - flavin containing dimethylaniline monoxygenase 3 Gene

FMO3 - flavin containing dimethylaniline monoxygenase 3 Gene

Homo sapiens

Also known as TMAU; FMOII; dJ127D3.1

Gene ID: 2328 | Gene type: protein coding

About FMO3

Cytogenetic location: 1q24.3 Genomic coordinates (GRCh38): 1:171,090,905-171,117,819 (from NCBI)

This gene has 6 transcripts (splice variants), 131 orthologues, 5 paralogues and is associated with 3 phenotypes. Biased expression in liver (RPKM 241.9) and adrenal (RPKM 8.6).

Summary

Flavin-containing monooxygenases (FMO) are an important class of drug-metabolizing Enzymes that catalyze the NADPH-dependent oxygenation of various nitrogen-,sulfur-, and phosphorous-containing xenobiotics such as therapeutic drugs, dietary compounds, pesticides, and Other foreign compounds. The human FMO gene family is composed of 5 genes and multiple pseudogenes. FMO members have distinct developmental- and tissue-specific expression patterns. The expression of this FMO3 gene, the major FMO expressed in adult liver, can vary up to 20-fold between individuals. This inter-individual variation in FMO3 expression levels is likely to have significant effects on the rate at which xenobiotics are metabolised and, therefore, is of considerable interest to the pharmaceutical industry. This transmembrane protein localizes to the endoplasmic reticulum of many tissues. Alternative splicing of this gene results in multiple transcript variants encoding different isoforms. Mutations in this gene cause the disorder trimethylaminuria (TMAu) which is characterized by the accumulation and excretion of unmetabolized trimethylamine and a distinctive body odor. In healthy individuals, trimethylamine is primarily converted to the non odorous trimethylamine N-oxide.[provided by RefSeq, Jan 2016]

FMO3 Products(4)

mRNA Protein Name
NM_001002294.3 NP_001002294.1 dimethylaniline monooxygenase [N-oxide-forming] 3 isoform a
NM_001319173.2 NP_001306102.1 dimethylaniline monooxygenase [N-oxide-forming] 3 isoform b
NM_001319174.2 NP_001306103.1 dimethylaniline monooxygenase [N-oxide-forming] 3 isoform c
NM_006894.6 NP_008825.4 dimethylaniline monooxygenase [N-oxide-forming] 3 isoform a
Gene Ontology
  • Molecular Function
  • Biological Process
Molecular Function GO Annotation Evidence Reference Source
enables hypotaurine monooxygenase activity IDA
IDA: Inferred from direct assay
32156684 GOA
enables protein binding IPI
IPI: Inferred from physical interaction
25910212 GOA
Biological Process GO Annotation Evidence Reference Source
involved in taurine biosynthetic process IDA
IDA: Inferred from direct assay
32156684 GOA
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

FMO3 Protein Structure

FMO-like

FMO-like: Flavin-binding monooxygenase-like (3 - 531)

  • 0
  • 100
  • 200
  • 300
  • 400
  • 500
  • 532 a.a.
Protein Preferred Names Protein Names

dimethylaniline monooxygenase [N-oxide-forming] 3

FMO form 2

FMO3 Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method Reference
Intra
FMO3 P31513 CLEC10A Homo sapiens Q8IUN9
Y2H Array
32296183
Intra
FMO3 P31513 CLEC10A Homo sapiens Q8IUN9
Y2H Prey Pooling
32296183
Intra
FMO3 P31513 CLEC10A Homo sapiens Q8IUN9
Validated Y2H
32296183
Intra
FMO3 P31513 SACM1L Homo sapiens Q9NTJ5
Validated Y2H
32296183
Intra
FMO3 P31513 CREB3 Homo sapiens O43889-2
Y2H Array
25910212
Intra
FMO3 P31513 CREB3 Homo sapiens O43889-2
Y2H Bait-Prey Pool
25910212
Intra
FMO3 P31513 CREB3 Homo sapiens O43889-2
Validated Y2H
25910212
Intra
FMO3 P31513 CREB3 Homo sapiens O43889-2
Y2H Array
31515488
Intra
FMO3 P31513 SGSM3 Homo sapiens Q96HU1
Validated Y2H
32296183
Intra
FMO3 P31513 SGSM3 Homo sapiens Q96HU1
Y2H Array
32296183
Intra
FMO3 P31513 SGSM3 Homo sapiens Q96HU1
Y2H Prey Pooling
32296183
Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases Alias
Trimethylaminuria

TMAU

Fish-Odor Syndrome

Fish Malodor Syndrome

Fish Odor Syndrome

Stale Fish Syndrome

Tmauria

Severe Primary Trimethylaminuria

Mesh

D008661

Fish Odour Syndrome

Primary Trimethylaminuria

Tmauria

Fish Odor Syndrome

Fmo3 Deficiency

Tmau

Trimethylaminuria

Familial Adenomatous Polyposis

Adenomatous Polyposis Coli

Fap

Familial Polyposis Coli

Familial Multiple Polyposis Syndrome

Adenomatous Polyposis Of The Colon

Familial Intestinal Polyposis

Fpc

Familial Adenomatous Polyposis Of The Colon

Familial Multiple Polyposis

Familial Polyposis Of The Colon

Hereditary Polyposis Coli

Polyposis, Adenomatous Intestinal

Adenomatous Familial Polyposis

Adenomatous Familial Polyposis Syndrome

Myh-Associated Polyposis

Colorectal Adenomatous Polyposis

Adenomatous Polyposis, Familial

Mutyh-Associate Polyposis

Sudden Infant Death Syndrome

SIDS

Sudden Infant Death Syndrome, Susceptibility To

Cot Death

Crib Death

Sudden Death Of Nonspecific Cause In Infancy

Sudden Infant Death

Death, Sudden, Syndrome, Infant

Choline Deficiency Disease

Choline Deficiency

Arteriosclerosis

Arteriosclerotic Vascular Disease

Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Orthologs Information

Species Symbol Source ID
Bos taurus FMO3 VGNC VGNC:29051
Mus musculus FMO3 MGD MGI:1100496
Canis familiaris FMO3 VGNC VGNC:40918
Rattus norvegicus FMO3 RGD RGD:619761
Macaca mulatta FMO3 VGNC VGNC:99953
Felis catus FMO3 VGNC VGNC:62309