Abstract
These disorders cause an unpleasant fish-like body odour. The problems are psychosocial and management centres on attempting to minimise the odour.
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References
Tjoa S, Fennessey P (1991) The identification of trimethylamine excess in man: quantitative analysis and biological origins, Anal Biochem 197:77–82
Al-Waiz M, Ayesh R, Mitchell SC et al (1987) A genetic polymorphism of the N-oxidation of trimethylamine in humans. Clin Pharmacol Ther 42:588–594
Ayesh R, Mitchell SC, Zhang A, Smith RL (1993) The fish odour syndrome: biochemical, familial, and clinical aspects. Br Med J 307:655–657
Mitchell SC, Smith RL (2001) Trimethylaminuria: the fish malodor syndrome. Drug Metab Dispos 29:517–521
Cashman JR, Zhang J (2002) Interindividual differences of human flavin-containing monooxygenase 3: genetic polymorphisms and functional variation. Drug Metab Dispos 30:1043–1052
Ziegler DM (1990) Flavin-containing monooxygenases: enzymes adapted for multisubstrate specificity (Review). Trends Pharmacol Sci 11:321–324
Koukouritaka SB, Simpson P, Yeung CK et al (2002) Human hepatic flavin-containing monooxygenase 1 (FMO1) and 3 (FMO3) developmental expression. Pediatr Res 51:236–243
Lee CWG, Yu JS, Turner BB, Murray KE (1976) Trimethylaminuria: fishy odours in children. N Engl J Med 295:937–938
Al-Waiz M, Ayesh R, Mitchell SC et al (1989) Trimethylaminuria: the detection of carriers using a trimethylamine load test. J Inherit Metab Dis 12:80–85
Pike MG, King GS, Pettit BR et al (1988) Lactulose in trimethylaminuria, the fish-odour syndrome. Helv Paediatr Acta 43:345–348
Shephard EA, Dolphin CT, Fox MF et al (1993) Localization of genes encoding three distinct flavin-containing monooxygenases to human chromosome1q. Genomics 16:85–89
Dolphin CT, Riley JH, Smith RL et al (1997) Structural organization of the human flavin-containing monooxygenase 3 gene (FMO3), the favored candidate for fish odor syndrome, determined directly from genomic DNA. Genomics 46:260–267
Hernandez D, Addou S, Lee D et al (2003) Trimethylaminuria and a human FMO3 mutation database. Hum Mutat 22:209–213
Treacy EP, Akerman BR, Chow LML et al (1998) Mutations of the flavin-containing monooxygenase gene (FMO3) cause trimethyl-aminuria, a defect in detoxication. Hum Mol Genet 7:839–845
Walker V (1993) The fish odour syndrome (leader). Br Med J 307:639–640
Moolenaar SH, Poggi-Bach J, Engelke UFH et al (1999) Defect in dimethylglycine dehydrogenase, a new inborn error of metabolism: NMR spectroscopy study. Clin Chem 45:459–464
Binzak BA, Vockley JG, Jenkins RB, Vockley J (2000) Structure and analysis of the human dimethylglycine dehydrogenase gene. Mol Genet Metab 69:181–187
Binzak BA, Wevers RA, Moolenaar SH et al (2001) Cloning of dimethylglycine dehydrogenase and a new human inborn error of metabolism, dimethylglycine dehydrogenase deficiency. Am J Hum Genet 68:839–847
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© 2006 Springer Medizin Verlag Heidelberg
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Walker, V., Wevers, R.A. (2006). Trimethylaminuria and Dimethylglycine Dehydrogenase Deficiency. In: Fernandes, J., Saudubray, JM., van den Berghe, G., Walter, J.H. (eds) Inborn Metabolic Diseases. Springer, Berlin, Heidelberg . https://doi.org/10.1007/978-3-540-28785-8_31
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DOI: https://doi.org/10.1007/978-3-540-28785-8_31
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