1. Gene
  2. FMO1 - flavin containing dimethylaniline monoxygenase 1 Gene

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

FMO1 Protein Structure

FMO-like

FMO-like: Flavin-binding monooxygenase-like (2 - 531)

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  • 532 a.a.
Protein Preferred Names Protein Names

dimethylaniline monooxygenase [N-oxide-forming] 1

FMO 1

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

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