FMO1: Flavin Containing Dimethylaniline Monoxygenase 1

A key enzyme in xenobiotic metabolism and drug detoxification

Gene Information Card

Symbol FMO1
Full Name Flavin Containing Dimethylaniline Monoxygenase 1
Gene Type protein-coding
Chromosomal Location 1q24.3
NCBI Gene ID 2326 ncbi.nlm.nih.gov/gene/2326
Ensembl ID ENSG00000010932
UniProt ID Q01740
OMIM ID 136130
HGNC ID 3776
Aliases FMO1, FMO 1, dimethylaniline monooxygenase [N-oxide-forming] 1

Description

FMO1 encodes a member of the flavin-containing monooxygenase (FMO) family, which catalyzes the oxygenation of nucleophilic nitrogen, sulfur, phosphorus, and selenium atoms in a wide variety of xenobiotics, including drugs, pesticides, and dietary compounds. FMO1 is predominantly expressed in fetal liver and adult kidney, and plays a critical role in the detoxification of foreign chemicals. The enzyme uses NADPH and FAD as cofactors and is involved in the metabolism of drugs such as itopride, sulindac sulfide, and benzydamine.

Disease Associations

Disease category Pathophysiological mechanism Genomic evidence
Trimethylaminuria (TMAU) FMO1 dysfunction may contribute to impaired trimethylamine oxidation, though FMO3 is the primary gene implicated. PMID: 10677296
Drug-induced liver injury Altered FMO1 activity can affect the metabolism of hepatotoxic drugs, potentially increasing susceptibility. PMID: 19536777
Hypertension Polymorphisms in FMO1 have been associated with altered metabolism of endogenous amines and blood pressure regulation. PMID: 21533021

Expression Profile

Tissue Expression
Tissue nTPM level
Kidney 12.5 High
Liver (fetal) 8.2 Medium
Adrenal gland 4.1 Low
Small intestine 3.0 Low
Lung 1.5 Not detected
Cell Line Expression
Cell Line nTPM Notes
HEK293 15.3 High expression in recombinant systems
HepG2 2.1 Low endogenous expression
Caco-2 1.8 Low expression
A549 0.5 Not detected
Data source:Human Protein Atlas(proteinatlas.org)

Mutations & Variants

Hotspot Mutations
Variant Type Frequency Functional Description
c.943G>A (p.Gly315Ser) Missense 0.01% Reduced catalytic activity in vitro
c.1285C>T (p.Arg429Cys) Missense 0.005% Decreased enzyme stability
c.1A>G (p.Met1Val) Start loss <0.001% Loss of protein expression
Mutation functional classification

Loss of Function (LOF)

Missense variants such as p.Gly315Ser and p.Arg429Cys reduce or abolish FMO1 enzymatic activity.

Gain of Function (GOF)

No gain-of-function mutations have been reported for FMO1.

Dominant Negative (DN)

No dominant-negative mutations have been described for FMO1.

Pathways

Drug metabolism - other enzymes (KEGG: hsa00983)
Metabolism of xenobiotics by cytochrome P450 (KEGG: hsa00980)
Chemical carcinogenesis (KEGG: hsa05204)

Protein Summary

FMO1 is a 532-amino acid microsomal flavoprotein that catalyzes the NADPH- and oxygen-dependent oxidation of soft nucleophilic heteroatoms in xenobiotics. The enzyme contains a conserved FAD- and NADPH-binding domain and is anchored to the endoplasmic reticulum membrane via an N-terminal transmembrane helix. FMO1 exhibits broad substrate specificity and is important for the detoxification of drugs and environmental chemicals. Its expression is developmentally regulated, with high levels in fetal liver and adult kidney, and it is not inducible by typical xenobiotic inducers.

Related Products

Product name Cat.No. Species Gene ID
FMO1 Knockout HEK293 Cell Line EDJ-KQ4614 Human 2326 Details Get a Quote
FMO1 Knockout HeLa Cell Line EDJ-KQ53253 Human 2326 Details Get a Quote
FMO1 Knockout A-549 Cell Line EDJ-KQ61735 Human 2326 Details Get a Quote
FMO1 Knockout HCT 116 Cell Line EDJ-KQ70222 Human 2326 Details Get a Quote
Displaying Records 1 To 4 Of 4 Records
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