FMO1 (flavin containing dimethylaniline monoxygenase 1)

Gene

• Entrez ID: 2326
• Gene name: Flavin containing dimethylaniline monoxygenase 1
• Gene symbol: FMO1
• Synonyms (NCBI Gene): -
• Chromosome: 1
• Chromosome location: 1q24.3
• 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

miRNA

miRTarBase ID	miRNA	Experiments	Reference
MIRT044436	hsa-miR-320a	CLASH	23622248
MIRT2534084	hsa-miR-4464	CLIP-seq
MIRT2534085	hsa-miR-4748	CLIP-seq
MIRT2534084	hsa-miR-4464	CLIP-seq
MIRT2534085	hsa-miR-4748	CLIP-seq

Gene ontology (GO)

GO ID	Ontology	Definition	Evidence	Reference
GO:0000098	Process	Sulfur amino acid catabolic process	TAS
GO:0004497	Function	Monooxygenase activity	IDA	19262426
GO:0004497	Function	Monooxygenase activity	IEA
GO:0004497	Function	Monooxygenase activity	TAS	1712018
GO:0004499	Function	N,N-dimethylaniline monooxygenase activity	IBA
GO:0004499	Function	N,N-dimethylaniline monooxygenase activity	IDA	15144220
GO:0004499	Function	N,N-dimethylaniline monooxygenase activity	IEA
GO:0005515	Function	Protein binding	IPI	25910212, 32296183
GO:0005783	Component	Endoplasmic reticulum	IEA
GO:0005788	Component	Endoplasmic reticulum lumen	TAS
GO:0005789	Component	Endoplasmic reticulum membrane	IEA
GO:0005789	Component	Endoplasmic reticulum membrane	ISS
GO:0006082	Process	Organic acid metabolic process	IDA	15144220
GO:0006805	Process	Xenobiotic metabolic process	IEA
GO:0009404	Process	Toxin metabolic process	IDA	15144220
GO:0016020	Component	Membrane	IEA
GO:0016491	Function	Oxidoreductase activity	IEA
GO:0032496	Process	Response to lipopolysaccharide	IEA
GO:0034899	Function	Trimethylamine monooxygenase activity	IEA
GO:0034899	Function	Trimethylamine monooxygenase activity	ISS
GO:0042412	Process	Taurine biosynthetic process	IBA
GO:0042412	Process	Taurine biosynthetic process	IDA	32156684
GO:0042412	Process	Taurine biosynthetic process	IEA
GO:0046322	Process	Negative regulation of fatty acid oxidation	IEA
GO:0047822	Function	Hypotaurine monooxygenase activity	EXP	32156684
GO:0047822	Function	Hypotaurine monooxygenase activity	IDA	32156684
GO:0047822	Function	Hypotaurine monooxygenase activity	IEA
GO:0050660	Function	Flavin adenine dinucleotide binding	IEA
GO:0050661	Function	NADP binding	IEA
GO:0097009	Process	Energy homeostasis	IEA

Other IDs

• MIM: 136130
• HGNC: 3769
• e!Ensembl: ENSG00000010932

Protein

• UniProt ID: Q01740
• Protein name: Flavin-containing monooxygenase 1 (EC 1.14.13.148) (EC 1.14.13.8) (Dimethylaniline monooxygenase [N-oxide-forming] 1) (Dimethylaniline oxidase 1) (Fetal hepatic flavin-containing monooxygenase 1) (FMO 1) (Trimethylamine monooxygenase)
• Protein function: Broad spectrum monooxygenase that catalyzes the oxygenation of a wide variety of nitrogen- and sulfur-containing compounds including xenobiotics (PubMed:32156684). Catalyzes the S-oxygenation of hypotaurine to produce taurine, an organic osmolyt
• Family and domains:

  Pfam

  Accession	ID	Position in sequence	Description	Type
  PF00743	FMO-like	2 → 532	Flavin-binding monooxygenase-like	Family

• Tissue specificity: TISSUE SPECIFICITY: Expressed mainly in fetal and adult liver. {ECO:0000269|PubMed:1712018}.
• Sequence:

  MAKRVAIVGAGVSGLASIKCCLEEGLEPTCFERSDDLGGLWRFTEHVEEGRASLYKSVVS
  NSCKEMSCYSDFPFPEDYPNYVPNSQFLEYLKMYANHFDLLKHIQFKTKVCSVTKCSDSA
  VSGQWEVVTMHEEKQESAIFDAVMVCTGFLTNPYLPLDSFPGINAFKGQYFHSRQYKHPD
  IFKDKRVLVIGMGNSGTDIAVEASHLAEKVFLSTTGGGWVISRIFDSGYPWDMVFMTRFQ
  NMLRNSLPTPIVTWLMERKINNWLNHANYGLIPEDRTQLKEFVLNDELPGRIITGKVFIR
  PSIKEVKENSVIFNNTSKEEPIDIIVFATGYTFAFPFLDESVVKVEDGQASLYKYIFPAH
  LQKPTLAIIGLIKPLGSMIPTGETQARWAVRVLKGVNKLPPPSVMIEEINARKENKPSWF
  GLCYCKALQSDYITYIDELLTYINAKPNLFSMLLTDPHLALTVFFGPCSPYQFRLTGPGK
  WEGARNAIMTQWDRTFKVIKARVVQESPSPFESFLKVFSFLALLVAIFLIFL

• Sequence length: 532

Pathways

KEGG:

Taurine and hypotaurine metabolism
Drug metabolism - cytochrome P450
Metabolic pathways

Reactome:

FMO oxidises nucleophiles

Associated diseases

Evidence Score:

• ★☆☆☆☆  Gene-disease association found in Text Mining only
• ★★☆☆☆  Found in Text Mining and Unknown/Other Associations
• ★★★☆☆  Reported in Unknown/Other Associations across ≥2 Sources
• ★★★★☆  ClinVar: Pathogenic/Likely Pathogenic (<5 Variants)
• ★★★★★  ClinVar: Pathogenic/Likely Pathogenic (≥5 Variants)

Unknown / Other Associations
Phenotype Name	Clinical Significance	Source	Evidence Score
AMYOTROPHIC LATERAL SCLEROSIS 1	—	CTD, Disgenet CTD, Disgenet	★★☆☆☆ Found in Text Mining + Unknown/Other Associations
AUTISM SPECTRUM DISORDER	—	CTD	★★☆☆☆ Found in Text Mining + Unknown/Other Associations
AUTISM SPECTRUM DISORDERS	—	Disgenet	★★☆☆☆ Found in Text Mining + Unknown/Other Associations
LIVER CIRRHOSIS, EXPERIMENTAL	—	CTD	★★☆☆☆ Found in Text Mining + Unknown/Other Associations
MOTOR NEURON DISEASE	—	Disgenet	★★☆☆☆ Found in Text Mining + Unknown/Other Associations
Nonpapillary renal cell carcinoma	Uncertain significance	ClinVar	★★☆☆☆ Found in Text Mining + Unknown/Other Associations

Associations from Text Mining
Disease Name	Disease (Merged)	Source	PMID	Relationship Type	Evidence Score
Amyotrophic Lateral Sclerosis	Amyotrophic Lateral Sclerosis	BEFREE	17127561, 21082301		★☆☆☆☆ Found in Text Mining only
AMYOTROPHIC LATERAL SCLEROSIS 1	Lateral Sclerosis	CTD_human_DG	17127561		★★☆☆☆ Found in Text Mining + Unknown/Other Associations
Amyotrophic Lateral Sclerosis, Familial	Amyotrophic lateral sclerosis	CTD_human_DG	17127561		★☆☆☆☆ Found in Text Mining only
Amyotrophic Lateral Sclerosis, Sporadic	Lateral Sclerosis	BEFREE	17127561		★☆☆☆☆ Found in Text Mining only
Amyotrophic Lateral Sclerosis, Sporadic	Lateral Sclerosis	CTD_human_DG	17127561		★☆☆☆☆ Found in Text Mining only
Color Blindness, Blue	Color blindness	BEFREE	20056567		★☆☆☆☆ Found in Text Mining only