FMO1 - flavin containing dimethylaniline monoxygenase 1 Gene

Homo sapiens

Gene ID: 2326 | Gene type: protein coding

About FMO1

Cytogenetic location: 1q24.3 Genomic coordinates (GRCh38): 1:171,248,494-171,285,978 (from NCBI)

This gene has 8 transcripts (splice variants), 137 orthologues and 5 paralogues. Biased expression in kidney (RPKM 54.7), small intestine (RPKM 8.2) and 1 other tissue.

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]

FMO1 Products(4)

mRNA	Protein	Name
NM_001282692.1	NP_001269621.1	dimethylaniline monooxygenase [N-oxide-forming] 1 isoform a
NM_001282693.2	NP_001269622.1	dimethylaniline monooxygenase [N-oxide-forming] 1 isoform b
NM_001282694.2	NP_001269623.1	dimethylaniline monooxygenase [N-oxide-forming] 1 isoform c
NM_002021.3	NP_002012.1	dimethylaniline monooxygenase [N-oxide-forming] 1 isoform b

Gene Ontology

Molecular Function
Biological Process

Molecular Function GO Annotation	Evidence	Reference	Source
enables N,N-dimethylaniline monooxygenase activity	IDA IDA: Inferred from direct assay	15144220	GOA
enables hypotaurine monooxygenase activity	EXP EXP: Inferred from Experiment	32156684	GOA
enables hypotaurine monooxygenase activity	IDA IDA: Inferred from direct assay	32156684	GOA
enables monooxygenase activity	IDA IDA: Inferred from direct assay	19262426	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	25910212	GOA

Biological Process GO Annotation	Evidence	Reference	Source
involved in NADPH oxidation	IDA IDA: Inferred from direct assay	15144220	GOA
involved in organic acid metabolic process	IDA IDA: Inferred from direct assay	15144220	GOA
involved in taurine biosynthetic process	IDA IDA: Inferred from direct assay	32156684	GOA
involved in toxin metabolic process	IDA IDA: Inferred from direct assay	15144220	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

FMO1 Protein Structure

FMO-like

0
100
200
300
400
500
532 a.a.

Protein Preferred Names

Protein Names

dimethylaniline monooxygenase [N-oxide-forming] 1

FMO 1

FMO1 Protein-protein interaction Information

Type Cross Intra	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	Reference
Intra	FMO1	Q01740	SLC10A6	Homo sapiens	Q3KNW5	Validated Y2H	32296183
Intra	FMO1	Q01740	SLC10A6	Homo sapiens	Q3KNW5	Y2H Array	32296183
Intra	FMO1	Q01740	SLC10A6	Homo sapiens	Q3KNW5	Y2H Prey Pooling	32296183
Intra	FMO1	Q01740	GRINA	Homo sapiens	Q7Z429	Validated Y2H	32296183
Intra	FMO1	Q01740	MFSD14B	Homo sapiens	Q5SR56	Y2H Prey Pooling	32296183
Intra	FMO1	Q01740	MFSD14B	Homo sapiens	Q5SR56	Y2H Array	32296183
Intra	FMO1	Q01740	MFSD14B	Homo sapiens	Q5SR56	Validated Y2H	32296183
Intra	FMO1	Q01740	CREB3	Homo sapiens	O43889-2	Y2H Array	25910212
Intra	FMO1	Q01740	CREB3	Homo sapiens	O43889-2	Validated Y2H	25910212
Intra	FMO1	Q01740	CREB3	Homo sapiens	O43889-2	Y2H Bait-Prey Pool	25910212
Intra	FMO1	Q01740	REEP4	Homo sapiens	Q9H6H4	Validated Y2H	32296183
Intra	FMO1	Q01740	REEP4	Homo sapiens	Q9H6H4	Y2H Array	32296183
Intra	FMO1	Q01740	REEP4	Homo sapiens	Q9H6H4	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

Thyroid Dyshormonogenesis 6

TDH6	Genetic Defect In Thyroid Hormonogenesis 6
Thyroid Hormonogenesis, Genetic Defect In, 6	Hypothyroidism, Congenital, Due To Dyshormonogenesis, 6
Chdh6	Congenital Hypothyroidism Due To Dyshormonogenesis Type 6

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

Related Agents

Pre-clinical Phase

Bioactive Molecules (2)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-P4439	H-Met-Val-OH		/	Unkown
HY-RS05005	FMO1 Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Canis familiaris	FMO1	VGNC	VGNC:40916
Macaca mulatta	FMO1	VGNC	VGNC:72682
Felis catus	FMO1	VGNC	VGNC:62307
Bos taurus	FMO1	VGNC	VGNC:29049
Rattus norvegicus	FMO1	RGD	RGD:2622
Mus musculus	FMO1	MGD	MGI:1310002

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