450:, such as red meat (beef, lamb and pork), liver, and offal. A study performed by Wang Z found that when comparing diets where the main protein source was red meat, white meat and non-meat protein sources, consumption of red meat increased the production of TMAO, whereas white meat and non-meat protein diets generated only low to negligible amounts TMAO. This study indicates that red meat is a major driver of TMA production by altering the balance of microbiota in the stomach due to the carnitine found in red meat. A further study by Crimarco A found that a 8 week plant based diet significantly reduced TMAO production, and further, that after switching the diet to include animal based protein, TMAO production was less than the participants who had only been given an animal protein based diet. The findings suggest that the microbiome in the gut is modified by a plant based diet, and for a time a person will lack the bacteria required to convert choline and carnitine into TMA at the same rate of an animal protein based diet.
461:, beans, whey products, milk, and other foods that contain high levels of choline. Choline is an essential nutrient so complete elimination of choline is unadvised. As above, white meat (chicken, turkey) and plant based products may be fine to consume if red meat is predominantly avoided. Note, while raw ingredients like soybeans have a relatively high choline content, some processed products like soy sauce, soy milk and tofu have low choline content, due to dilution of ingredients, small serving size, or removal as a byproduct during the manufacturing process. It's best to check the choline content of food and the portion size for a better understanding of how much choline is being consumed.
165:) is ingested. Some precursor is absorbed into the bloodstream in the small intestine before reaching the gut (the RDI of choline is 450–550 mg per day, which is absorbed this way), however there is a limit to the transport capacity of the intestine, and not all precursor is exposed to the process. Unabsorbed precursor ends up in the gut. Certain bacteria in the gut can convert those precursors to TMA, the proportion of precursor converted to TMA is related to the amount of specific bacteria in the gut but on average 63% of excess choline, and 31% of carnitine (±13%) are converted to TMA.
67:
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much TMA for the amount of enzyme, then TMA will continue to circulate in the bloodstream until enough enzyme is produced. While TMA is in the bloodstream, it is filtered out via the kidneys (95% over 24 hours) to the bladder, and slowly exits the body in bodily fluids; urine, sweat, saliva, reproductive fluids and breath. TMA has no known interactions with any known internal or organ function.
416:
schooling, personal life, career and relationships, resulting in social isolation, low self-esteem, depression, paranoid behaviour, and suicide. Delayed diagnosis, body odour and the lack of cure may lead to psychosocial issues. When the condition is suspected or known to occur in a family, genetic testing can be helpful in identifying the specific individuals who have or carry the disorder.
385:
emphasis is frequently placed on multiple consultations to reduce the risk of misdiagnosis, and also asking the individual to have a reliable confidant accompany them to the consultation who can confirm the reality of the reported symptom. ORS patients are unable to provide such confidants as they have no objective odor.
556:
and 31% of carnitine (+-13%) are converted to TMA by an average gut profile - even if dysbiosis raised these values to 100%, it would still not meet the thresholds as above for precursor overload on a regular diet. TMAU2 by gut dysbiosis is currently a hypothetical condition requiring further research.
564:
The first clinical case of TMAU was described in 1970. Her mother related that her child, a 6 year old girl, had intermittently had a peculiar "fishy" odour. Analysis of her urine showed an elevated level of trimethylamine, and that a chemically pure free base sample of trimethylamine smelled similar
419:
The metabolic and clinical manifestations of TMAU are generally regarded as benign, as there is no associated organ dysfunction. This designation, and the fact that the condition is often unrecognised by doctors, misdiagnosed and can have important ramifications including missed or delayed diagnosis.
411:
ORS-like paranoia, due to the potential lack of ability to smell the odour oneself and the worry that it generates. It is recommended to organise reliable confidants, colleagues, friends or relatives ("odor buddies") to work with the sufferer to discretely inform them if they are presenting an odour.
198:
Trimethylamine is also released in the person's sweat, reproductive fluids, and breath, and can give off a fishy odor when the concentration of trimethylamine is high enough to be detected. The intensity of the smell is directly correlated with the concentration of trimethylamine in the bloodstream.
555:
While the balance of gut bacteria does play a part in the amount of TMA being produced from precursors in the gut (antibiotic treatment temporarily stops odour), gut bacteria in general convert a significant proportion of dietary TMA precursors already. For example, on average 63% of excess choline,
169:
TMA in the gut is absorbed through the intestinal lining and enters the bloodstream, where it is processed by the liver. A healthy liver produces an abundance of the enzyme FMO3, which neutralises the TMA by oxidising it to an odourless TMAO. If FMO3 enzyme production is compromised, or there is too
206:
Smell events are often sporadic and episodic in nature (based on diet over the previous 24 hours), making it often difficult to diagnose by smell alone. Some people with trimethylaminuria report having a strong odor all the time, but there has not been any evidence apart from self reported symptoms
185:
are technically precursors to TMA, pilot studies have shown no significant effect on the production of excess TMA/TMAO in urinary analysis at normal dietary levels of consumption. When taken in large quantities (12g/day) betaine has been known to cause fish odor symptoms, meaning that there is some
520:
A fish-like body odor can result from extreme excess consumption of TMA precursors such as choline, carnitine and betaine (usually unobtainable via regular dietary intake, it requires high levels of supplement intake). 900mg of trimethylamine, 8g-20g of choline, 3g of carnitine or 20g betatine has
202:
People with TMAU may have an intermittent fish-like body odor, depending on diet and the severity of their FM03 mutation. In a study by Wise PM, of 115 positively identified TMAU subjects, after a choline challenge load test (intentionally ingesting a TMA precursor) only 10% expressed a smell at a
551:
in the gut. There is no reference to scientific research confirming gut dysbiosis alone can cause TMAU2, and this type of diagnosis was not produced at other testing sites. Cases were claimed to be successfully cured with antibiotics and dietary changes as above. In 2017 a new gas chromatography
242:
pattern, which means two copies of the gene in each cell are altered. The parents of an individual with an autosomal recessive disorder are both carriers of one copy of the altered gene. Carriers may have mild symptoms of trimethylaminuria or experience temporary episodes of fish-like body odor.
415:
Affected individuals experience shame and embarrassment, fail to maintain relationships, avoid contact with people who comment on their condition, and are obsessive about masking the odour with hygiene products and even smoking. The malodorous aspect can have serious and destructive effects on
384:
for TMAU were deemed likely to instead have ORS. Findings found that the use of “fecal/sewage” as a description, and the use of multiple descriptors of the smell, and 'incorrect' locations of smell origin effectively differentiated ORS from TMAU. In the literature on body odour identification,
431:
Diet and supplement use should be overseen by medical professionals and nutritionists, as dietary restrictions can have other serious adverse health effects. Choline in particular is an essential nutrient required for proper neural formation in foetal and childhood development, if pregnant or
300:
gene, trimethylamine is not broken down and instead builds up in the body. As the compound is released in a person's sweat, urine, and breath, it causes the strong odor characteristic of trimethylaminuria. Researchers believe that stress and diet also play a role in triggering symptoms.
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Lenherr N, Berndt A, Ritz N, Rudin C. Aerococcus urinae: a possible reason for malodorous urine in otherwise healthy children. Eur J Pediatr. 2014;173:1115-7; Gibb AP, Sivaraman B. A second case of foul smelling urine in a boy caused by
Aerococcus urinae. Pediatr Infect Dis J.
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Cashman JR, Camp K, Fakharzadeh SS, Fennessey PV, Hines RN, Mamer OA, Mitchell SC, Nguyen GP, Schlenk D, Smith RL, Tjoa SS, Williams DE, Yannicelli S (2003). "Biochemical and clinical aspects of the human flavin-containing monooxygenase form 3 (FMO3) related to trimethylaminuria".
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social distance. When in a fasted state (12 hours) 0% had a smell detectable at a social distance and only 5% had some minor malodour at an intimate distance. These findings suggested that those that produced an odour had a more severe form of FMO3 impairment.
951:"A randomized crossover trial on the effect of plant-based compared with animal-based meat on trimethylamine-N-oxide and cardiovascular disease risk factors in generally healthy adults: Study With Appetizing Plantfood-Meat Eating Alternative Trial (SWAP-MEAT)"
521:
been known to cause temporary TMAU-like fish odour symptoms. These symptoms only last until supplement intake has ceased. Note, the RDI for choline is 450-550mg and most typical diets fall below this value, so an excessive amount of precursor is required.
194:
Trimethylamine is most noticeable in urine, as it is captured, concentrated and released in intervals. Fishy smelling urine is a primary identifying symptom in infant children (Trimethylaminuria literally meaning "trimethylamine in urine").
217:
and estrogen aggravate the condition. According to several reports, the condition worsens around puberty. In women, symptoms may worsen just before and during menstrual periods, after taking oral contraceptives, and around menopause.
396:; methylmercaptan; dimethylsulfide) with each exhalation, creating a ‘malodorous cloud’ in their vicinity”. It is possible that other causes such as halitosis, haemorrhoids, regular bromhidrosis, ORS or in severe cases, a
1762:
1880:
Yamazaki H, Fujieda M, Togashi M, et al. (2004). "Effects of the dietary supplements, activated charcoal and copper chlorophyllin, on urinary excretion of trimethylamine in
Japanese trimethylaminuria patients".
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2362:
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Guo, Y., Hwang, L. D., Li, J., Eades, J., Yu, C. W., Mansfield, C., ... & Preti, G. (2017). Genetic analysis of impaired trimethylamine metabolism using whole exome sequencing. BMC medical genetics, 18(1),
494:-oxide (TMAO) metabolite. Study participants experienced subjective reduction in odor as well as objective reduction in TMA and increase in TMAO concentration measured in their urine. The study found that:
133:
is caused by genetic mutations that affect the FMO3 function of the liver. Symptoms matching TMAU can also occur when there is no genetic cause, yet excessive TMA is excreted - this has been described as
464:
Vitamin B2 at 50 mg per day in combination with diet resolved smell issues for 2 children with TMAU. B2 was found to increase residual FMO3 performance in the liver, meaning more TMA is neutralised.
41:
380:
is a condition where there is a persistent false belief and preoccupation with the idea of emitting an abnormal body odor. According to McNiven at a
Canadian genetics clinic, 83% of referrals for
1278:
Allerston CK, Shimizu M, Fujieda M, Shephard EA, Yamazaki H, Phillips IR (2007). "Molecular evolution and balancing selection in the flavin-containing monooxygenase 3 gene (FMO3)".
388:
Unrelated fecal smells are an often misinterpreted self reported symptom associated with TMAU, Cashman JR found that 53% of TMAU and 59% of non-TMAU subjects suffered from regular
331:
Measurement of urine for the ratio of trimethylamine to trimethylamine N-oxide is the standard screening test. A blood test is available to provide genetic analysis. The prominent
585:
newspapers in both print and Online, giving an interview about her experiences with
Trimethylaminuria. The article was later repurposed in media across the globe, most notably by
1546:
Mountain H, Brisbane JM, Hooper AJ, Burnett JR, Goldblatt J (2008). "Trimethylaminuria (fish malodour syndrome): a "benign" genetic condition with major psychosocial sequelae".
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to the patient's fishy odor. They tested her condition by giving her more trimethylamine, which substantially increased her odour (which it did not in control subjects).
949:
Crimarco A, Springfield S, Petlura C, Streaty T, Cunanan K, Lee J, Fielding-Singh P, Carter MM, Topf MA, Wastyk HC, Sonnenburg ED, Sonnenburg JL, Gardner CD (2020).
547:
Between the years of 1997 and 2017, Sheffield
Children's Hospital in England diagnosed several hundred people with TMAU2, and suggested the majority to be caused by
428:
There is no known permanent cure for primary trimethylaminuria, but symptoms can in most cases be managed via diet, and sometimes by certain supplements (as below).
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in order to reduce the amount of bacteria in the gut, although this is not recommended as a long term solution due to antibiotic resistance and other side effects.
1837:
Treacy E, Johnson D, Pitt JJ, Danks DM (1995). "Trimethylaminuria, fish odour syndrome: A new method of detection and response to treatment with metronidazole".
1390:
McNiven V, Mamane S, et al. (2019). "The Nose Knows… or Does it? Olfactory
Reference Syndrome in Patients Presenting for Assessment of Unusual Body Odor".
1117:
Wise PM, Eades J, Tjoa S, Fennessey PV, Preti G (2011). "Individuals reporting idiopathic malodor production: demographics and incidence of trimethylaminuria".
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When food is consumed that contains TMA and/or TMAO (predominately seafood; saltwater fish, shellfish, seaweed and kelp). TMAO is converted by bacteria in the
392:, caused by dental plaque on the back of the tongue, which produced on average "200-600 ppb of sulfurous/fecal smelling volatile sulfur compounds (i.e., VSC:
1225:
Furnes B, Feng J, Sommer SS, Schlenk D (2003). "Identification of novel variants of the flavin-containing monooxygenase gene family in
African Americans".
1712:"Impact of chronic dietary red meat, white meat, or non-meat protein on trimethylamine N-oxide metabolism and renal excretion in healthy men and women"
272:-containing compounds from the diet, including trimethylamine. These compounds are produced by bacteria in the intestine as they digest proteins from
490:
may temporarily improve the quality of life of individuals afflicted with TMAU by helping their bodies to oxidize and convert TMA to the odorless
2570:
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715:
Zschocke J, Kohlmueller D, Quak E, Meissner T, Hoffmann GF, Mayatepek E (1999). "Mild trimethylaminuria caused by common variants in FMO3 gene".
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is not working correctly or if not enough enzyme is produced, the body loses the ability to properly convert the fishy-smelling chemical
600:, which went on a journey to find an official diagnosis for the condition, and again sparked a global media interest in the condition.
1788:"Diagnosis and phenotypic assessment of trimethylaminuria, and its treatment with riboflavin: 1H NMR spectroscopy and genetic testing"
626:, features a character with trimethylaminuria, although the condition was first described in the year in which the movie takes place.
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A similar foul-smelling odor of the urine has also been associated with colonization of the urinary tract with a bacterium called
213:
The condition seems to be more common in women than men, for unknown reasons. Scientists suspect that such female sex hormones as
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machine and testing procedure was introduced, which has far fewer false positive results, and very few (if any) TMAU2 results.
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In the case where someone is experiencing liver failure or kidney failure, TMA is sometimes an element present as part of
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can occur in the following conditions, where elevated TMA can be present in the urine without any underlying TMAU:
1710:
Wang Z, Bergeron N, Levison BS, Li XS, Chiu S, Jia X, Koeth RA, Li Y, Wu Y, Tang WH, Krauss RM, Hazen SL (2019).
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Avoiding all seafood, including fish, shellfish, kelp, and seaweed. Sea life contains TMAO, which is used as an
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597:
532:. It is unknown if these cases were temporary or not as the individuals did not return for subsequent testing.
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377:
151:
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897:"Implication of Trimethylamine N-Oxide (TMAO) in Disease: Potential Biomarker or New Therapeutic Target"
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3217:
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92:
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mutations account for most known cases of trimethylaminuria, some cases are caused by other factors.
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Individuals with this condition do not have any physical symptoms, and they typically appear healthy.
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Trimethylamine then builds up and is released in the person's sweat, urine, and breath, giving off a
1679:
Demarquoy J, Georges B, Rigault C, Royer MC, Clairet A, Soty M, Lekounoungou S, Le Borgne F (2004).
846:"Structure and Function of CutC Choline Lyase from Human Microbiota Bacterium Klebsiella pneumoniae"
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which has no odor. If the enzyme is missing or its activity is reduced because of a mutation in the
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2532:
2403:
66:
2027:
3038:
2703:
1681:"Radioisotopic determination of l-carnitine content in foods commonly eaten in Western countries"
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349:
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Bouchemal N, Ouss L, Brassier A, Barbier V, Gobin S, Hubert L, de Lonlay P, Le Moyec L (2019).
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Kalnins G, Kuka J, Grinberga S, Makrecka-Kuka M, Liepinsh E, Dambrova M, Tars K (August 2015).
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If not pregnant or breastfeeding, reducing the consumption of foods and supplements containing
289:
126:
3187:
2622:
2053:"Riboflavin-responsive trimethylaminuria in a patient with homocystinuria on betaine therapy"
1052:"Riboflavin-Responsive Trimethylaminuria in a Patient with Homocystinuria on Betaine Therapy"
3048:
2998:
2753:
2510:
1643:
593:
112:
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Humbert JA, Hammond KB, Hathaway WE (1970). "Trimethylaminuria: the fish-odour syndrome".
8:
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There are more than 40 known mutations associated with TMAU. Loss-of-function mutations,
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2004:
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1953:
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1632:"Trimethylamine oxide accumulation as a function of depth in Hawaiian mid-water fishes"
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Trimethylamine enters the body via the consumption of certain foods and supplements:
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Additionally, at least one study has suggested that daily intake of the supplements
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are three of the most common. Nonsense and missense mutations cause the most severe
40:
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3095:
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2868:
2284:
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2222:"Fish odour syndrome with features of both primary and secondary trimethylaminuria"
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Fraser-Andrews EA, Manning NJ, Ashton GH, Eldridge P, McGrath J, Menagé Hd (2003).
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gene was studied, including the evolution of some mutations associated with TMAU.
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1918:"Trimethylaminuria: the detection of carriers using a trimethylamine load test"
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Mitchell SC, Smith RL (2001). "Trimethylaminuria: the fish malodor syndrome".
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1995:
1980:"Trimethylaminuria: causes and diagnosis of a socially distressing condition"
1941:
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1499:
1085:
1028:
787:"Treatments of trimethylaminuria: where we are and where we might be heading"
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605:
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85% of test participants experienced complete loss of detectable "fishy" odor
487:
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Using slightly acidic detergent and body washes with a pH between 5.5 and 6.5
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407:
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When a food substance, supplement or medicine that contains a TMA precursor (
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1464:(11th ed.). St. Louis, Mo.: Elsevier/Saunders. pp. 1333, 1334.
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610:
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Hernandez D, Addou S, Lee D, Orengo C, Shephard EA, Phillips IR (2003).
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Shephard EA, Treacy, Eileen P, Phillips, Ian R (30 November 2011).
1050:
Manning NJ, Allen EK, Kirk RJ, Sharrard MJ, Smith EJ (2011-11-20).
586:
573:
In 2014, singer/songwriter Cassie Graves was first featured in the
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269:
246:
174:
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2645:
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2562:
2326:"'I Smell Like Rotting Fish': Woman Has Rare Fish Odour Syndrome"
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948:
895:
Janeiro MH, Ramírez MJ, Milagro FI, Martínez JA, Solas M (2018).
843:
458:
454:
186:
conversion of betaine to TMA if supplements are taken regularly.
182:
158:
2397:
1978:
Mackay RJ, McEntyre CJ, Henderson C, Lever M, George PM (2011).
226:
2804:
2523:
2366:
1484:
1433:
Richter JL (Apr 1996). "Diagnosis and treatment of halitosis".
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714:
446:
Reducing the consumption of foods and supplements that contain
265:
96:
1460:
Newman MG, Takei HH, Klokkevold PR, Carranza FA, eds. (2012).
524:
Two cases of the disorder have been identified in adults with
2051:
Manning NJ, Allen EK, Kirk RJ, Sharrard MJ, Smith EJ (2012).
1545:
1459:
894:
525:
238:
Most cases of trimethylaminuria appear to be inherited in an
2144:"TMAU – diagnostic testing at Sheffield Children's Hospital"
1977:
1785:
1678:
1005:"Dietary precursors of trimethylamine in man: a pilot study"
2201:"UK TMAU Test Stats 2017-2021 : Activist uses FOI act"
1916:
al-Waiz M, Ayesh R, Mitchell SC, Idle JR, Smith RL (1989).
277:
253:
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1523:
Trimethylaminuria (TMAU): history, diagnosis and genetics
281:
2050:
1915:
1049:
672:"Mutations of the flavin-containing monooxygenase gene (
2312:"Rare condition leaves singer smelling of rotting fish"
1636:
Deep Sea
Research Part I: Oceanographic Research Papers
1224:
1175:
2274:
1879:
1836:
1178:"Trimethylaminuria and a human FMO3 mutation database"
1116:
111:(TMA) from precursor compounds in food digestion into
1709:
432:
breast-feeding a low choline diet should be avoided.
2344:
2161:"Sheffield TMAU test back : And new ref ranges"
676:) cause trimethylaminuria, a defect in detoxication"
95:
that causes a defect in the normal production of an
1330:"Clinical utility gene card for: Trimethylaminuria"
504:
5% did not experience any detectable odor reduction
288:enzyme converts fishy-smelling trimethylamine into
2457:This article incorporates public domain text from
1002:
2630:
501:10% experienced some reduction in detectable odor
3271:
1478:
457:- fish, red meat, white meat, offal, egg yolks,
2571:3-hydroxy-3-methylglutaryl-CoA lyase deficiency
1588:
1435:Compendium of Continuing Education in Dentistry
1323:
1321:
1319:
1317:
2957:6-Pyruvoyltetrahydropterin synthase deficiency
508:
2483:
2268:
2181:"Comment on the NEW UK TMAU Test (Sheffield)"
1830:
1389:
1385:
1383:
784:
642:
613:features a character with trimethylaminuria.
435:Ways of reducing the fishy odor may include:
2841:2-Methylbutyryl-CoA dehydrogenase deficiency
2463:The National Human Genome Research Institute
1873:
1629:
1582:
1314:
1003:Zhang AQ, Mitchell SC, Smith RL (May 1999).
780:
778:
3139:Carbamoyl phosphate synthetase I deficiency
2576:3-Methylcrotonyl-CoA carboxylase deficiency
2028:"Office of Dietary Supplements - Carnitine"
1426:
443:to counter hydrostatic pressure underwater.
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1453:
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542:
65:
2119:"Office of Dietary Supplements - Choline"
2094:
2003:
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1589:Kloster I, Erichsen MM (September 2021).
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912:
888:
871:
861:
812:
802:
775:
759:"Office of Dietary Supplements - Choline"
691:
2309:
467:Taking low doses of antibiotics such as
225:
2818:Isobutyryl-CoA dehydrogenase deficiency
1703:
1432:
939:
3272:
2644:
1922:Journal of Inherited Metabolic Disease
1779:
1150:
1148:
669:
335:for TMA N-oxygenation is coded by the
3154:Ornithine transcarbamylase deficiency
3149:N-Acetylglutamate synthase deficiency
2471:
2459:The U.S. National Library of Medicine
2226:Clinical and Experimental Dermatology
1973:
1971:
1969:
1967:
1757:
1755:
592:In 2016, Graves was then featured in
189:
3071:Dopamine beta hydroxylase deficiency
1441:(4): 370–2, 374–6 passim, quiz 388.
998:
996:
994:
256:, which is found on the long arm of
141:
2907:Methylmalonyl-CoA mutase deficiency
2141:
1630:Bockus AB, Seibel BA (2016-06-01).
1145:
13:
1964:
1752:
1560:10.5694/j.1326-5377.2008.tb02126.x
1462:Carranza's clinical periodontology
1334:European Journal of Human Genetics
1156:"Learning About Trimethylaminuria"
404:may be the cause of fecal smells.
14:
3301:
2659:Glutathione synthetase deficiency
2340:
991:
284:, and other foods. Normally, the
115:(TMAO), through a process called
101:flavin-containing monooxygenase 3
2238:10.1046/j.1365-2230.2003.01230.x
1984:The Clinical Biochemist. Reviews
568:
364:Advanced liver or kidney disease
39:
2318:
2303:
2213:
2193:
2173:
2153:
2135:
2111:
2044:
2020:
1909:
1672:
1623:
1514:
1370:
1271:
1261:
1218:
1169:
1110:
1043:
850:Journal of Biological Chemistry
670:Treacy EP, et al. (1998).
260:, cause trimethylaminuria. The
2962:Tetrahydrobiopterin deficiency
1697:10.1016/j.foodchem.2003.09.023
837:
785:Schmidt AC, Leroux JC (2020).
751:
708:
663:
636:
1:
3285:Autosomal recessive disorders
3207:Lysinuric protein intolerance
2581:3-Methylglutaconic aciduria 1
2289:10.1016/S0140-6736(70)90241-2
1021:10.1016/s0278-6915(99)00028-9
729:10.1016/S0140-6736(99)80019-1
629:
319:In 2007 the evolution of the
2846:Beta-ketothiolase deficiency
2310:Reporter MN (July 2, 2014).
1404:10.1097/NMD.0000000000000933
1292:10.1097/FPC.0b013e328256b198
1131:10.1016/j.amjmed.2011.05.030
1009:Food and Chemical Toxicology
804:10.1016/j.drudis.2020.06.026
596:' Medical Mysteries on UK's
539:, the "breath of the dead".
423:
378:Olfactory reference syndrome
326:
152:lower gastrointestinal tract
7:
3280:Inborn errors of metabolism
3249:Ethylmalonic encephalopathy
509:Secondary Trimethylaminuria
221:
136:secondary trimethylaminuria
10:
3306:
3239:2-Hydroxyglutaric aciduria
3218:Oculocerebrorenal syndrome
559:
374:, especially in children.
3244:Aminoacylase 1 deficiency
3231:
3173:
3134:Argininosuccinic aciduria
3103:
3093:
3057:
3043:Hermansky–Pudlak syndrome
3011:
2970:
2947:
2934:
2924:
2887:
2859:
2851:Maple syrup urine disease
2831:
2823:Maple syrup urine disease
2803:
2788:
2762:
2739:
2711:
2696:
2621:
2599:
2591:Maple syrup urine disease
2561:
2522:
2509:
2429:
2348:
1895:10.1016/j.lfs.2003.10.022
1805:10.1186/s13023-019-1174-6
1763:"Choline : USDA ARS"
1657:10.1016/j.dsr.2016.03.005
230:Trimethylaminuria has an
131:Primary trimethylaminuria
54:
47:
38:
33:Primary trimethylaminuria
28:
23:
2533:Glutaric acidemia type 1
2409:C536561 C536561, C536561
1728:10.1093/eurheartj/ehy799
1500:10.2174/1389200033489505
680:Human Molecular Genetics
3039:Oculocutaneous albinism
1608:10.4045/tidsskr.21.0142
863:10.1074/jbc.M115.670471
543:TMAU2 and gut dysbiosis
350:Urinary tract infection
234:pattern of inheritance.
207:that this is the case.
3158:translocase deficiency
2902:Methylmalonic acidemia
2684:Glycine encephalopathy
1716:European Heart Journal
1595:Tidsskr Nor Laegeforen
1280:Pharmacogenet Genomics
235:
3188:Solute carrier family
2503:amino acid metabolism
1346:10.1038/ejhg.2011.214
229:
89:fish malodor syndrome
3049:Waardenburg syndrome
2999:Tyrosinemia type III
2754:Prolidase deficiency
2069:10.1007/8904_2011_99
1239:10.1124/dmd.31.2.187
1068:10.1007/8904_2011_99
967:10.1093/ajcn/nqaa203
594:Princess Productions
113:trimethylamine oxide
3254:Fumarase deficiency
2994:Tyrosinemia type II
2654:D-Glyceric acidemia
2609:Hypertryptophanemia
2586:Isovaleric acidemia
1839:J Inherit Metab Dis
1792:Orphanet J Rare Dis
1648:2016DSRI..112...37B
1591:"Trimethylaminuria"
856:(35): 21732–21740.
814:20.500.11850/423705
693:10.1093/hmg/7.5.839
355:Bacterial vaginosis
240:autosomal recessive
232:autosomal recessive
2989:Tyrosinemia type I
2912:Propionic acidemia
2879:Hypermethioninemia
2548:Pipecolic acidemia
2430:External resources
1934:10.1007/BF01805534
1851:10.1007/bf00710420
1195:10.1002/humu.10252
914:10.3390/nu10101398
651:(4 Pt 2): 517–21.
484:activated charcoal
333:enzyme responsible
310:missense mutations
306:nonsense mutations
236:
190:Symptoms and signs
93:metabolic disorder
85:fish odor syndrome
3267:
3266:
3259:Trimethylaminuria
3169:
3168:
3165:
3164:
3089:
3088:
3085:
3084:
2920:
2919:
2784:
2783:
2731:Urocanic aciduria
2692:
2691:
2617:
2616:
2453:
2452:
2444:Trimethylaminuria
2078:978-3-642-28095-5
1889:(22): 2739–2747.
1526:, 8 February 2013
1471:978-1-4377-0416-7
1227:Drug Metab Dispos
1125:(11): 1058–1063.
1077:978-3-642-28095-5
791:Drug Discov Today
645:Drug Metab Dispos
528:damage caused by
398:bowel obstruction
371:Aerococcus urinae
268:that breaks down
142:Metabolic pathway
83:), also known as
77:Trimethylaminuria
74:
73:
24:Trimethylaminuria
18:Medical condition
3297:
3214:Fanconi syndrome
3101:
3100:
3077:Brunner syndrome
2945:
2944:
2932:
2931:
2869:Cystathioninuria
2801:
2800:
2709:
2708:
2642:
2641:
2628:
2627:
2520:
2519:
2492:
2485:
2478:
2469:
2468:
2346:
2345:
2334:
2333:
2322:
2316:
2315:
2307:
2301:
2300:
2272:
2266:
2265:
2217:
2211:
2210:
2208:
2207:
2197:
2191:
2190:
2188:
2187:
2177:
2171:
2170:
2168:
2167:
2157:
2151:
2150:
2148:
2139:
2133:
2132:
2130:
2129:
2115:
2109:
2108:
2098:
2048:
2042:
2041:
2039:
2038:
2024:
2018:
2017:
2007:
1975:
1962:
1961:
1913:
1907:
1906:
1877:
1871:
1870:
1834:
1828:
1827:
1817:
1807:
1798:(1): 14(1):222.
1783:
1777:
1776:
1774:
1773:
1767:www.ars.usda.gov
1759:
1750:
1749:
1739:
1707:
1701:
1700:
1676:
1670:
1669:
1659:
1627:
1621:
1620:
1610:
1586:
1580:
1579:
1543:
1534:
1533:
1532:
1531:
1518:
1512:
1511:
1482:
1476:
1475:
1457:
1451:
1450:
1430:
1424:
1423:
1387:
1378:
1374:
1368:
1367:
1357:
1325:
1312:
1311:
1275:
1269:
1265:
1259:
1258:
1222:
1216:
1215:
1197:
1173:
1167:
1166:
1164:
1162:
1152:
1143:
1142:
1114:
1108:
1107:
1097:
1047:
1041:
1040:
1000:
989:
988:
978:
961:(5): 1188–1199.
946:
937:
936:
926:
916:
892:
886:
885:
875:
865:
841:
835:
834:
816:
806:
797:(9): 1710–1717.
782:
773:
772:
770:
769:
755:
749:
748:
712:
706:
705:
695:
667:
661:
660:
640:
394:hydrogen sulfide
70:
69:
43:
21:
20:
3305:
3304:
3300:
3299:
3298:
3296:
3295:
3294:
3270:
3269:
3268:
3263:
3227:
3202:Iminoglycinuria
3197:Hartnup disease
3178:
3161:
3111:
3081:
3053:
3029:Ocular albinism
3007:
2966:
2949:Phenylketonuria
2916:
2883:
2855:
2827:
2794:
2780:
2758:
2749:Hyperprolinemia
2735:
2704:α-ketoglutarate
2702:
2688:
2679:GAMT deficiency
2613:
2595:
2557:
2553:Saccharopinuria
2526:/straight chain
2505:
2496:
2454:
2449:
2448:
2425:
2424:
2357:
2343:
2338:
2337:
2332:. July 3, 2014.
2324:
2323:
2319:
2308:
2304:
2283:(7676): 770–1.
2273:
2269:
2218:
2214:
2205:
2203:
2199:
2198:
2194:
2185:
2183:
2179:
2178:
2174:
2165:
2163:
2159:
2158:
2154:
2146:
2140:
2136:
2127:
2125:
2117:
2116:
2112:
2079:
2049:
2045:
2036:
2034:
2026:
2025:
2021:
1976:
1965:
1914:
1910:
1878:
1874:
1835:
1831:
1784:
1780:
1771:
1769:
1761:
1760:
1753:
1708:
1704:
1677:
1673:
1628:
1624:
1587:
1583:
1544:
1537:
1529:
1527:
1520:
1519:
1515:
1488:Curr Drug Metab
1483:
1479:
1472:
1458:
1454:
1431:
1427:
1392:J Nerv Ment Dis
1388:
1381:
1377:2013;32:1300-1.
1375:
1371:
1326:
1315:
1276:
1272:
1266:
1262:
1223:
1219:
1174:
1170:
1160:
1158:
1154:
1153:
1146:
1115:
1111:
1078:
1048:
1044:
1001:
992:
947:
940:
893:
889:
842:
838:
783:
776:
767:
765:
757:
756:
752:
723:(9181): 834–5.
713:
709:
668:
664:
641:
637:
632:
624:Alexander Payne
603:The 2010 novel
571:
562:
545:
537:Fetor hepaticus
511:
426:
382:genetic testing
360:Cervical cancer
343:False positives
329:
290:trimethylamine
224:
192:
154:(gut) into TMA.
144:
64:
19:
12:
11:
5:
3303:
3293:
3292:
3287:
3282:
3265:
3264:
3262:
3261:
3256:
3251:
3246:
3241:
3235:
3233:
3229:
3228:
3226:
3225:
3220:
3210:
3209:
3204:
3199:
3194:
3184:
3182:
3171:
3170:
3167:
3166:
3163:
3162:
3160:
3151:
3146:
3141:
3136:
3131:
3126:
3124:
3123:
3122:
3109:Hyperammonemia
3098:
3091:
3090:
3087:
3086:
3083:
3082:
3080:
3079:
3073:
3067:
3065:
3063:Norepinephrine
3055:
3054:
3052:
3051:
3046:
3036:
3021:
3019:
3009:
3008:
3006:
3005:
2996:
2991:
2986:
2976:
2974:
2968:
2967:
2965:
2964:
2959:
2953:
2951:
2942:
2929:
2922:
2921:
2918:
2917:
2915:
2914:
2909:
2904:
2898:
2896:
2885:
2884:
2882:
2881:
2876:
2874:Homocystinuria
2871:
2865:
2863:
2857:
2856:
2854:
2853:
2848:
2843:
2837:
2835:
2829:
2828:
2826:
2825:
2820:
2815:
2813:Hypervalinemia
2809:
2807:
2798:
2786:
2785:
2782:
2781:
2779:
2778:
2772:
2770:
2760:
2759:
2757:
2756:
2751:
2745:
2743:
2737:
2736:
2734:
2733:
2728:
2723:
2717:
2715:
2706:
2694:
2693:
2690:
2689:
2687:
2686:
2681:
2667:
2666:
2661:
2656:
2650:
2648:
2639:
2625:
2619:
2618:
2615:
2614:
2612:
2611:
2605:
2603:
2597:
2596:
2594:
2593:
2588:
2583:
2578:
2573:
2567:
2565:
2559:
2558:
2556:
2555:
2550:
2545:
2543:Hyperlysinemia
2540:
2535:
2529:
2527:
2517:
2507:
2506:
2495:
2494:
2487:
2480:
2472:
2451:
2450:
2447:
2446:
2434:
2433:
2431:
2427:
2426:
2423:
2422:
2411:
2400:
2389:
2374:
2358:
2353:
2352:
2350:
2349:Classification
2342:
2341:External links
2339:
2336:
2335:
2317:
2302:
2267:
2232:(2): 203–205.
2212:
2192:
2172:
2152:
2134:
2123:ods.od.nih.gov
2110:
2077:
2043:
2032:ods.od.nih.gov
2019:
1963:
1908:
1872:
1845:(3): 306–312.
1829:
1778:
1751:
1722:(7): 583–594.
1702:
1691:(1): 137–142.
1685:Food Chemistry
1671:
1622:
1581:
1535:
1513:
1494:(2): 151–170.
1477:
1470:
1452:
1425:
1398:(3): 145–151.
1379:
1369:
1313:
1286:(10): 827–39.
1270:
1260:
1217:
1168:
1144:
1109:
1076:
1042:
1015:(5): 515–520.
990:
955:Am J Clin Nutr
938:
887:
836:
774:
763:ods.od.nih.gov
750:
707:
662:
634:
633:
631:
628:
622:, directed by
616:The 2023 film
570:
567:
561:
558:
544:
541:
510:
507:
506:
505:
502:
499:
480:
479:
476:
465:
462:
451:
444:
425:
422:
402:fecal vomiting
366:
365:
362:
357:
352:
328:
325:
264:gene makes an
223:
220:
191:
188:
167:
166:
155:
143:
140:
109:trimethylamine
72:
71:
58:
52:
51:
49:Trimethylamine
45:
44:
36:
35:
30:
26:
25:
17:
9:
6:
4:
3:
2:
3302:
3291:
3290:Rare diseases
3288:
3286:
3283:
3281:
3278:
3277:
3275:
3260:
3257:
3255:
3252:
3250:
3247:
3245:
3242:
3240:
3237:
3236:
3234:
3230:
3224:
3221:
3219:
3215:
3212:
3211:
3208:
3205:
3203:
3200:
3198:
3195:
3193:
3189:
3186:
3185:
3183:
3181:
3176:
3172:
3159:
3155:
3152:
3150:
3147:
3145:
3144:Citrullinemia
3142:
3140:
3137:
3135:
3132:
3130:
3127:
3125:
3120:
3117:
3116:
3115:
3110:
3106:
3102:
3099:
3097:
3092:
3078:
3074:
3072:
3069:
3068:
3066:
3064:
3060:
3056:
3050:
3047:
3044:
3040:
3037:
3034:
3030:
3026:
3023:
3022:
3020:
3018:
3014:
3010:
3004:
3003:Hawkinsinuria
3000:
2997:
2995:
2992:
2990:
2987:
2985:
2981:
2978:
2977:
2975:
2973:
2969:
2963:
2960:
2958:
2955:
2954:
2952:
2950:
2946:
2943:
2941:
2937:
2936:Phenylalanine
2933:
2930:
2928:
2923:
2913:
2910:
2908:
2905:
2903:
2900:
2899:
2897:
2895:
2891:
2886:
2880:
2877:
2875:
2872:
2870:
2867:
2866:
2864:
2862:
2858:
2852:
2849:
2847:
2844:
2842:
2839:
2838:
2836:
2834:
2830:
2824:
2821:
2819:
2816:
2814:
2811:
2810:
2808:
2806:
2802:
2799:
2797:
2792:
2791:propionyl-CoA
2787:
2777:
2774:
2773:
2771:
2769:
2765:
2761:
2755:
2752:
2750:
2747:
2746:
2744:
2742:
2738:
2732:
2729:
2727:
2724:
2722:
2719:
2718:
2716:
2714:
2710:
2707:
2705:
2700:
2695:
2685:
2682:
2680:
2676:
2672:
2669:
2668:
2665:
2662:
2660:
2657:
2655:
2652:
2651:
2649:
2647:
2643:
2640:
2638:
2634:
2629:
2626:
2624:
2620:
2610:
2607:
2606:
2604:
2602:
2598:
2592:
2589:
2587:
2584:
2582:
2579:
2577:
2574:
2572:
2569:
2568:
2566:
2564:
2560:
2554:
2551:
2549:
2546:
2544:
2541:
2539:
2536:
2534:
2531:
2530:
2528:
2525:
2521:
2518:
2516:
2512:
2508:
2504:
2500:
2493:
2488:
2486:
2481:
2479:
2474:
2473:
2470:
2466:
2465:
2464:
2460:
2445:
2441:
2440:
2436:
2435:
2432:
2428:
2421:
2417:
2416:
2412:
2410:
2406:
2405:
2401:
2399:
2395:
2394:
2390:
2388:
2384:
2383:
2379:
2375:
2373:
2369:
2368:
2364:
2360:
2359:
2356:
2351:
2347:
2331:
2327:
2321:
2313:
2306:
2298:
2294:
2290:
2286:
2282:
2278:
2271:
2263:
2259:
2255:
2251:
2247:
2243:
2239:
2235:
2231:
2227:
2223:
2216:
2202:
2196:
2182:
2176:
2162:
2156:
2145:
2138:
2124:
2120:
2114:
2106:
2102:
2097:
2092:
2088:
2084:
2080:
2074:
2070:
2066:
2062:
2058:
2054:
2047:
2033:
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2980:Alkaptonuria
2796:succinyl-CoA
2726:Histidinemia
2721:Carnosinemia
2664:Sarcosinemia
2499:Inborn error
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2057:JIMD Reports
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2972:Tyrosinemia
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2142:Manning N.
611:Ned Beauman
486:and copper
400:leading to
29:Other names
3274:Categories
3223:Cystinosis
3192:Cystinuria
3105:Urea cycle
2984:Ochronosis
2861:Methionine
2833:Isoleucine
2601:Tryptophan
2515:acetyl-CoA
2415:DiseasesDB
2206:2023-10-02
2186:2023-10-02
2166:2023-10-02
2128:2023-10-02
2037:2023-10-02
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1119:Am. J. Med
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630:References
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575:Daily Mail
314:phenotypes
179:creatinine
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120:-oxidation
3180:IE of RTT
3175:Transport
3119:aspartate
3075:reverse:
2768:glutamine
2764:Glutamate
2713:Histidine
2699:glutamate
2246:0307-6938
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1942:0141-8955
1666:0967-0637
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598:Channel 5
549:dysbiosis
530:hepatitis
513:Although
448:carnitine
424:Treatment
390:halitosis
327:Diagnosis
247:Mutations
173:Although
163:carnitine
56:Specialty
3114:arginine
3059:Tyrosine
3025:Albinism
3013:Tyrosine
2940:tyrosine
2927:fumarate
2888:General
2675:Creatine
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2105:23430919
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1883:Life Sci
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583:Metro UK
469:neomycin
441:osmolyte
270:nitrogen
222:Genetics
175:lecithin
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2646:Glycine
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