Flavin-containing monooxygenase

The flavin-containing monooxygenase (FMO) protein family consists of a group of enzymes that catalyze chemical reactions via the bound cofactor flavin. These reactions involve oxidation of heteroatoms, particularly nucleophilic atoms such as the nitrogen of amines.[1]

The best-known such protein is called FMO3 and is mutated in the vast majority of cases of trimethylaminuria, a genetic disease that causes deficiencies in breakdown of trimethylamine and gives the patient a fishy body odor.[2] In yeast, FMO proteins are associated with redox cycling of glutathione to glutathione disulfide, a system that maintains the redox state of the cell and heavily influences the protein folding rates of disulfide bond-containing proteins.[3]

## Genes

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* FMO1, FMO2, FMO3, FMO4, FMO5

## References

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1. ↑ Cashman JR. (1995). Structural and catalytic properties of the mammalian flavin-containing monooxygenase. Chem Res Toxicol 8(2):166-81. PMID 7766799
2. ↑ Hernandez D, Addou S, Lee D, Orengo C, Shephard EA, Phillips IR (2003). Trimethylaminuria and a human FMO3 mutation database. Hum Mutat 22 (3): 209-13. PMID 12938085
3. ↑ Suh JK, Poulsen LL, Ziegler DM, Robertus JD. (1999). Yeast flavin-containing monooxygenase generates oxidizing equivalents that control protein folding in the endoplasmic reticulum. Proc Natl Acad Sci 96(6):2687-91. PMID 10077572

## External links

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* flavin-containing+monooxygenase at the US National Library of Medicine Medical Subject Headings (MeSH)
* EC 1.14.13.8

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Oxidoreductases: dioxygenases, including steroid hydroxylases (EC 1.14)

1.14.11: 2-oxoglutarate|

* Prolyl hydroxylase
* Lysyl hydroxylase

1.14.13: NADH or NADPH|

* Flavin-containing monooxygenase
* FMO1
* FMO2
* FMO3
* FMO4
* FMO5
* Nitric oxide synthase
* NOS1
* NOS2
* NOS3
* Cholesterol 7 alpha-hydroxylase
* Methane monooxygenase
* 3A4
* Lanosterol 14 alpha-demethylase
* 24-hydroxycholesterol 7α-hydroxylase

1.14.14: reduced flavin or flavoprotein|

* 19A1
* 2D6
* 2E1

1.14.15: reduced iron-sulfur protein|

* 11B1
* 11B2
* 11A1

1.14.16: reduced pteridine (BH4 dependent)|

* Phenylalanine hydroxylase
* Tyrosine hydroxylase
* Tryptophan hydroxylase

1.14.17: reduced ascorbate|

* Dopamine beta-hydroxylase

1.14.18-19: other|

* Tyrosinase
* Stearoyl-CoA desaturase-1

1.14.99 \- miscellaneous|

* Cyclooxygenase
* Heme oxygenase (HMOX1)
* Squalene monooxygenase
* 17A1
* 21A2

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