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The Journal of the Royal Institute of Thailand Vol. 27 No. 1 Jan.-Mar. 2002 ‚√§°≈‘ Ë πµ— «‡À¡Á π ÒÚÙ Abstract Fish-Malodor Syndrome Amnuay Thithapandha Faculty of Science, Mahidol University, Bangkok 10700, Thailand The fish-malodor syndrome (also known as the fish-odor syndrome and trimethylaminuria) is a metabolic disorder characterized by the presence of abnormal amounts of the dietary-derived ter- tiary amine, trimethylamine, contained in urine, perspiration, expired air, and other bodily secre- tions. Trimethylamine itself has the powerful aroma of rotting fish, and this confers upon the suf- ferer a highly objectionable body odor, which can be destructive to the personal, social, and work life of the affected individual. The disease itself is transmitted in a Mandelian fashion as an autoso- mal recessive trait. In recent years, much progress has been made at all levels – clinical, epidemio- logical, biochemical, and genetic – in our understanding of this unfortunate condition. The present article summarizes this progress and highlights the current needs in the treatment of such patients. Key words : fish-malodor syndrome, fish-odor syndrome, trimethylaminuria specificity of trimethylamine N-oxy- genation by human flavin-contain- ing monooxygenase (FMO) and P450 enzymes: selective catalysis by FMO3. Biochem Pharmacol 1998; 56: 1005-12. ÒÛ. Lee CW, Tomlinson B, Yeung JHK, Lin G, Damani LA. Distribution of the N-oxidation of dietary-derived trimethylamine in a male Chinese population. Pharmacogenetics 2000; 10: 1-3. ÒÙ. Mitchell SC. The fish-odor syn- drome. Perspect Biol Med 1996; 39: 514-26. Òı. Mitchell SC, Zhang AQ, Barrett T, Ayesh R, Smith RL. Studies on the discontinuous N-oxidation of trim- ethylamine among Jordanian, Ecua- dorian and New Guinean populations. Pharmacogenetics 1997; 7: 45-50. Òˆ. Mitchell SC, Smith RL. Trimethyla- minuria: the fish-malodor syndrome. Drug Metab Disp 2001; 29: 517-21. Ò˜. Murphy HC, Dolphin CT, Jamo- hamed A, Holmes HC, Michelakakis H, Shephard EA, et al. A novel mu- tation in the flavin-containing mono- oxygenase 3 gene, FMO3, that causes fish-odor syndrome: activity of the mutant enzyme assessed by proton NMR spectroscopy. Pharmacogenet- ics 2000; 10: 439-51. Ò¯. Shelley ED, Shelley WD. The fish- odor syndrome - trimethylaminuria. JAMA 1984; 251: 253-5. Ò˘. Õ”π«¬ ∂‘ ∞“æ— π∏å . A pharmacogenetic study of trimethylaminuria in orien- tals. Pharmacogenetics 1997; 7: 497- 501. Ú. Treacy EP, Akerman B, Chow LML, Youri R, Bibeau C, Lin J, et al. Mutations of the flavin-containing monooxygenase gene (FMO3) cause trimethylaminuria, a defect in detoxi- cation. Hum Mol Genet 1998; 7: 839-45. ÚÒ. Zhang AQ, Mitchell SC, Ayesh R, Smith RL. Determination of tri- methylamine and related aliphatic amines in human urine by head-space gas chromatography. J Chromatogr 1992; 584: 141-5. ÚÚ. Zhang AQ, Mitchell SC, Smith RL. Discontinuous distribution of N-oxi- dation of dietary-derived trimethyl- amine in a British population. Xenobiotica 1996; 26: 957-61.