HomeAboutRankingsData Sources
© 2026 GeneE
🧬
GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
ⓘGeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
FMO1
flavin containing dimethylaniline monoxygenase 1
Chromosome 1 · 1q24.3
NCBI Gene: 2326Ensembl: ENSG00000010932.17HGNC: HGNC:3769UniProt: B2RCG5
48PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
organic acid metabolic processN,N-dimethylaniline monooxygenase activityprotein bindingmonooxygenase activityhypertrophic cardiomyopathymultinodular goiterBlackfan-Diamond anemiaHereditary persistence of fetal hemoglobin - beta-thalassemia
✦AI Summary

FMO1 (flavin-containing monooxygenase 1) is a broad-spectrum monooxygenase that catalyzes the oxygenation of nitrogen- and sulfur-containing compounds, including xenobiotics and endogenous metabolites 1. It catalyzes S-oxygenation of hypotaurine to produce taurine, which regulates cell volume and provides cytoprotective functions 1. FMO1 also catalyzes N-oxygenation of trimethylamine (TMA) to trimethylamine N-oxide (TMAO), potentially detoxifying this gut microbiota-derived metabolite 1. In human tissues, FMO1 shows marked developmental and tissue-specific expression: negligible in adult liver but highly expressed in kidney (47±9 pmol/mg) and fetal liver (14.4±3.5 pmol/mg), indicating it functions primarily as an extrahepatic drug-metabolizing enzyme in adults 2. Within skin, FMO1 marks a major fibroblast population (larger cells in interstitial and perivascular locations) distinct from SFRP2+ fibroblasts, suggesting roles in tissue structure and homeostasis 3. Disease relevance includes TMAO-mediated metabolic dysfunction, as TMAO activates the ER stress kinase PERK, driving metabolic syndrome progression 4. FMO1 metabolizes pesticides including phorate and fenthion to their sulfoxide metabolites 5, indicating its clinical significance in xenobiotic detoxification and drug metabolism across renal and intestinal tissues.

Sources cited
1
FMO1 is a broad-spectrum monooxygenase catalyzing oxygenation of nitrogen/sulfur-containing compounds and xenobiotics; catalyzes S-oxygenation of hypotaurine to taurine and N-oxygenation of TMA to TMAO
PMID: 32156684
2
FMO1 protein quantification across human tissues showing high expression in kidney and fetal liver but minimal in adult liver; demonstrates functional expression in intestine metabolizing sulfides stereoselectively
PMID: 10950857
3
FMO1 defines a major fibroblast population in human skin with larger cells in interstitial and perivascular locations, distinct from SFRP2+ fibroblasts
PMID: 29080679
4
TMAO (product of FMO-catalyzed TMA oxygenation) is a gut-microbe-derived metabolite associated with metabolic syndrome; activates PERK kinase to promote metabolic dysfunction
PMID: 31543404
5
FMO1 catalyzes monooxygenation of pesticides phorate, fenthion, and methiocarb to their respective sulfoxide metabolites with defined kinetic parameters
PMID: 36446195
Disease Associationsⓘ20
hypertrophic cardiomyopathyOpen Targets
0.27Weak
multinodular goiterOpen Targets
0.11Weak
Blackfan-Diamond anemiaOpen Targets
0.08Suggestive
Hereditary persistence of fetal hemoglobin - beta-thalassemiaOpen Targets
0.07Suggestive
hereditary persistence of fetal hemoglobin-sickle cell disease syndromeOpen Targets
0.07Suggestive
inosine triphosphatase deficiencyOpen Targets
0.07Suggestive
alpha thalassemia-intellectual disability syndrome type 1Open Targets
0.07Suggestive
Alpha-thalassemia - intellectual disability syndrome linked to chromosome 16Open Targets
0.07Suggestive
hemoglobin D diseaseOpen Targets
0.06Suggestive
Autosomal dominant methemoglobinemiaOpen Targets
0.06Suggestive
delta-beta-thalassemiaOpen Targets
0.06Suggestive
Hemoglobin E - beta-thalassemiaOpen Targets
0.06Suggestive
hemoglobin E-beta-thalassemia syndromeOpen Targets
0.06Suggestive
hemolytic anemia due to adenylate kinase deficiencyOpen Targets
0.05Suggestive
autosomal dominant sideroblastic anemiaOpen Targets
0.05Suggestive
Constitutional sideroblastic anemiaOpen Targets
0.05Suggestive
Heinz body anemiaOpen Targets
0.05Suggestive
Alpha-thalassemia - myelodysplastic syndromeOpen Targets
0.05Suggestive
alpha-thalassemia-myelodysplastic syndromeOpen Targets
0.05Suggestive
hemoglobin C-beta-thalassemia syndromeOpen Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CYP3A4Protein interaction98%CYP2C9Protein interaction93%CYP2D6Protein interaction93%LOC107987478Protein interaction93%LOC107987479Protein interaction93%FMO4Co-mentioned in literature60%
Tissue Expression6 tissues
Ovary
100%
Heart
26%
Lung
12%
Liver
9%
Bone Marrow
8%
Brain
3%
Gene Interaction Network
Click a node to explore
FMO1CYP3A4CYP2C9CYP2D6LOC107987478LOC107987479FMO4
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q01740
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.89LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.62 [0.44–0.89]
RankingsWhere FMO1 stands among ~20K protein-coding genes
  • #9,074of 20,598
    Most Researched48
  • #7,928of 17,882
    Most Constrained (LOEUF)0.89
Genes detectedFMO1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
SFRP2/DPP4 and FMO1/LSP1 Define Major Fibroblast Populations in Human Skin.
PMID: 29080679
J Invest Dermatol · 2018
1.00
2
FMO2 ameliorates nonalcoholic fatty liver disease by suppressing ER-to-Golgi transport of SREBP1.
PMID: 37874228
Hepatology · 2025
0.90
3
Trimethylamine N-Oxide Binds and Activates PERK to Promote Metabolic Dysfunction.
PMID: 31543404
Cell Metab · 2019
0.80
4
Flavin-containing monooxygenase 2 confers cardioprotection in ischemia models through its disulfide bond catalytic activity.
PMID: 39480513
J Clin Invest · 2024
0.70
5
Immunoquantitation of FMO1 in human liver, kidney, and intestine.
PMID: 10950857
Drug Metab Dispos · 2000
0.60