Article
Trimethylaminuria and a human FMO3 mutation database.
Human mutation - 1 Sept 2003
Hernandez Diana, Addou Sarah, Lee David, Orengo Christine, Shephard Elizabeth A, Phillips Ian R
Abstract excerpt
Trimethylaminuria (TMAuria), or fish-odor syndrome, is due to defective flavin-containing monooxygenase 3 (FMO3). In the liver, this protein catalyzes the NADPH-dependent oxidative metabolism of odorous trimethylamine (TMA), derived in the gut from dietary sources, to nonodorous trimethylamine N-oxide (TMA N-oxide). Affected individuals are unable to carry out this reaction and consequently exude a fishy body...
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