Gene: [01q2/FMO3] flavin containing monooxygenase 3 (adult liver); trimethylaminuria (fish-odor syndrome; MIM:602079);

FUN

[1] Systematic name: N,N-dimethylaniline,NADPH:oxygen oxidoreductase (N-oxide forming).
[2] The reaction catalyzed: N,N-dimethylaniline + NADPH + O(2) = = N,N-dimethylaniline N-oxide + NADP(+) + H(2)O.
[3] Cofactor: FAD."

PAT

Trimethylaminuria, otherwise known as fish odor syndrome, is an inborn error of metabolism associated with an offensive body odor and caused by deficiency of FMO-mediated N-oxidation of amino-trimethylamine (TMA) derived from foodstuffs. The causative mutation resides in FMO3 (Akerman-1997). Mutant FMO3 cDNA for this mutation demonstrated loss of substrate activity for TMA and tyramine."

REF

PAT,MUT "Akerman BR &: AJHG, 61, (Suppl), 1876-1876, 1997
REV,PAT,TER "Mitchell SC: Perspect Biol Med, 39, 514-526, 1996
PRO,LOC,FAG "Shephard EA &: Genomics, 16, 85-89, 1993

KEY

xen, mtbd

CLA

coding, basic

LOC

01 q23-25

MIM

MIM: 136132

EZN

ENZYME: 1.14.13.8

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