Materiały źródłowe

• #1 A Review of Trimethylaminuria: (Fish Odor Syndrome)

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3848652/

  The mechanism involves the destruction of gut bacteria, which are responsible for the reduction of TMAO into TMA. […] TMAU is most commonly an inherited disorder that is characterized by an unpleasant odor similar to that of rotting fish. The disease itself is not life threatening, but it significantly diminishes the quality of patients lives, and has even been associated with suicide attempts. […] There are many options to reduce the severity of the condition, which range from simple lifestyle changes, such as washing with acidic soap and eating a choline-deficient diet to utilizing antibiotics and sequestering agents.

• #2 Trimethylaminuria – Wikipedia

  https://en.wikipedia.org/wiki/Trimethylaminuria

  Trimethylaminuria (TMAU), also known as fish odor syndrome or fish malodor syndrome, is a rare metabolic disorder that causes a defect in the normal production of an enzyme named flavin-containing monooxygenase 3 (FMO3). When FMO3 is not working correctly or if not enough enzyme is produced, the body loses the ability to properly convert the fishy-smelling chemical trimethylamine (TMA) from precursor compounds in food digestion into trimethylamine oxide (TMAO), through a process called N-oxidation. […] Trimethylamine then builds up and is released in the person’s sweat, urine, and breath, giving off a fishy odor. Primary trimethylaminuria is caused by genetic mutations that affect the FMO3 function of the liver. […] Mutations in the FMO3 gene, which is found on the long arm of chromosome 1, cause trimethylaminuria. The FMO3 gene makes an enzyme that breaks down nitrogen-containing compounds from the diet, including trimethylamine.

• #3 Trimethylaminuria – Wikipedia

  https://en.wikipedia.org/wiki/Trimethylaminuria

  Normally, the FMO3 enzyme converts fishy-smelling trimethylamine into trimethylamine N-oxide which has no odor. If the enzyme is missing or its activity is reduced because of a mutation in the FMO3 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. […] There are more than 40 known mutations associated with TMAU. Loss-of-function mutations, nonsense mutations, and missense mutations are three of the most common. Nonsense and missense mutations cause the most severe phenotypes. […] 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.

• #4 Trimethylaminuria – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK594255/

  Trimethylaminuria (TMAU) is a rare metabolic disorder resulting in the accumulation of trimethylamine. Primary TMAU is an autosomal recessive genetic condition that results in the deficiency or dysfunction of the hepatic enzyme flavin monooxygenase 3. The underlying pathogenesis of TMAU is usually a deficient or dysfunctional hepatic enzyme, flavin-containing monooxygenase 3 (FMO3). This enzyme metabolizes TMA into an odorless compound, trimethylamine N-oxide (TMAO. Primary trimethylaminuria occurs secondary to a genetic mutation of the FMO3 gene, located on chromosome 1q24.3. Various FMO3 mutations result in decreased enzyme activity, impaired substrate binding, or disrupted protein structure. Failure to metabolize TMA results in its accumulation; it is subsequently excreted via urine, sweat, breath, and other body secretions. The excess excretion is what causes the fishy odor. Primary TMAU is caused by a dysfunction or deficiency of the flavin-containing monooxygenase 3 (FMO3) enzyme due to mutations in FMO3. The FMO3 enzyme plays a crucial role in the metabolism of TMA, a compound with a fishy odor. The dysfunctional or deficient FMO3 enzyme in patients with primary TMAU impairs TMA conversion, resulting in an increased TMA:TMAO ratio. The accumulated TMA is passively absorbed into the bloodstream and is released through various excretory routes, including sweat, breath, and urine, giving rise to the distinctive fishy odor associated with TMAU. The gut microbiota produces TMA by metabolizing certain nitrogen-containing compounds, such as choline and carnitine, in certain foods. In individuals with TMAU, the excessive production of TMA by colonic bacteria further contributes to the accumulation of TMA in the body.

• #5 A Review of Trimethylaminuria: (Fish Odor Syndrome)

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3848652/

  Trimethylaminuria, better known as fish odor syndrome, is a psychologically disabling condition in which a patient emits a foul odor, which resembles that of rotting fish. The disorder is most commonly caused by an inherited deficiency in flavin monooxygenase 3, the vital enzyme for the metabolism of trimethylamine, which is the compound responsible for the unpleasant odor. […] The cause of the syndrome is rooted in the dysfunctional metabolism of TMA, which is normally oxidized by flavin monooxygenase 3 (FMO3) into non-odorous trimethylamine-N-oxide (TMAO). […] Most patients with FOS are eventually diagnosed with primary trimethylaminuria, which is caused by a deficiency in FMO3 that is inherited in an autosomal recessive fashion. Under normal circumstances, dietary TMAO and other TMA precursors, such as choline, are ingested and then reduced to TMA by colonic bacteria.

• #6 Trimethylaminuria

  https://dermnetnz.org/topics/trimethylaminuria

  Trimethylaminuria is also known as fish (mal)odour syndrome because of the characteristic fishy body odour. […] Trimethylamine is a volatile aliphatic molecule, best known as the smell of rotting fish. […] Bacteria in the bowel produce the malodourous trimethylamine from trimethylamine N-oxide or choline. […] Normally it is converted to the odourless trimethylamine N-oxide by an enzyme in the liver, known as trimethylamine oxidase or flavin-containing mono-oxygenase 3 (FMO3). […] In trimethylaminuria, this malodourous molecule is excreted in sweat, urine, breath, saliva, vaginal and other body secretions. […] Primary trimethylaminuria is a rare autosomal recessive genetic disease (MIM 602079), meaning the affected person has inherited two copies of the defective gene, one from each parent.

• #7 Trimethylaminuria – Wikipedia

  https://en.wikipedia.org/wiki/Trimethylaminuria

  Trimethylaminuria (TMAU), also known as fish odor syndrome or fish malodor syndrome, is a rare metabolic disorder that causes a defect in the normal production of an enzyme named flavin-containing monooxygenase 3 (FMO3). When FMO3 is not working correctly or if not enough enzyme is produced, the body loses the ability to properly convert the fishy-smelling chemical trimethylamine (TMA) from precursor compounds in food digestion into trimethylamine oxide (TMAO), through a process called N-oxidation. […] Trimethylamine then builds up and is released in the person’s sweat, urine, and breath, giving off a fishy odor. Primary trimethylaminuria is caused by genetic mutations that affect the FMO3 function of the liver. […] Mutations in the FMO3 gene, which is found on the long arm of chromosome 1, cause trimethylaminuria. The FMO3 gene makes an enzyme that breaks down nitrogen-containing compounds from the diet, including trimethylamine.

• #8 Trimethylaminuria – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK594255/

  Trimethylaminuria (TMAU) is a rare metabolic disorder resulting in the accumulation of trimethylamine. Primary TMAU is an autosomal recessive genetic condition that results in the deficiency or dysfunction of the hepatic enzyme flavin monooxygenase 3. The underlying pathogenesis of TMAU is usually a deficient or dysfunctional hepatic enzyme, flavin-containing monooxygenase 3 (FMO3). This enzyme metabolizes TMA into an odorless compound, trimethylamine N-oxide (TMAO. Primary trimethylaminuria occurs secondary to a genetic mutation of the FMO3 gene, located on chromosome 1q24.3. Various FMO3 mutations result in decreased enzyme activity, impaired substrate binding, or disrupted protein structure. Failure to metabolize TMA results in its accumulation; it is subsequently excreted via urine, sweat, breath, and other body secretions. The excess excretion is what causes the fishy odor. Primary TMAU is caused by a dysfunction or deficiency of the flavin-containing monooxygenase 3 (FMO3) enzyme due to mutations in FMO3. The FMO3 enzyme plays a crucial role in the metabolism of TMA, a compound with a fishy odor. The dysfunctional or deficient FMO3 enzyme in patients with primary TMAU impairs TMA conversion, resulting in an increased TMA:TMAO ratio. The accumulated TMA is passively absorbed into the bloodstream and is released through various excretory routes, including sweat, breath, and urine, giving rise to the distinctive fishy odor associated with TMAU. The gut microbiota produces TMA by metabolizing certain nitrogen-containing compounds, such as choline and carnitine, in certain foods. In individuals with TMAU, the excessive production of TMA by colonic bacteria further contributes to the accumulation of TMA in the body.

• #9 Trimethylaminuria – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK594255/

  Trimethylaminuria (TMAU) is a rare metabolic disorder resulting in the accumulation of trimethylamine. Primary TMAU is an autosomal recessive genetic condition that results in the deficiency or dysfunction of the hepatic enzyme flavin monooxygenase 3. The underlying pathogenesis of TMAU is usually a deficient or dysfunctional hepatic enzyme, flavin-containing monooxygenase 3 (FMO3). This enzyme metabolizes TMA into an odorless compound, trimethylamine N-oxide (TMAO. Primary trimethylaminuria occurs secondary to a genetic mutation of the FMO3 gene, located on chromosome 1q24.3. Various FMO3 mutations result in decreased enzyme activity, impaired substrate binding, or disrupted protein structure. Failure to metabolize TMA results in its accumulation; it is subsequently excreted via urine, sweat, breath, and other body secretions. The excess excretion is what causes the fishy odor. Primary TMAU is caused by a dysfunction or deficiency of the flavin-containing monooxygenase 3 (FMO3) enzyme due to mutations in FMO3. The FMO3 enzyme plays a crucial role in the metabolism of TMA, a compound with a fishy odor. The dysfunctional or deficient FMO3 enzyme in patients with primary TMAU impairs TMA conversion, resulting in an increased TMA:TMAO ratio. The accumulated TMA is passively absorbed into the bloodstream and is released through various excretory routes, including sweat, breath, and urine, giving rise to the distinctive fishy odor associated with TMAU. The gut microbiota produces TMA by metabolizing certain nitrogen-containing compounds, such as choline and carnitine, in certain foods. In individuals with TMAU, the excessive production of TMA by colonic bacteria further contributes to the accumulation of TMA in the body.

• #10 Trimethylaminuria

  https://dermnetnz.org/topics/trimethylaminuria

  Two defective copies of the gene result in a failure to produce sufficient active FMO3 enzyme. […] Some mutations cause a reduction in FMO3 enzyme activity and others result in complete loss of enzyme activity. […] Secondary trimethylaminuria occurs when the liver FMO3 enzyme is either overwhelmed or underactive for some reason. […] The enzyme may be overwhelmed by an excessive dietary intake of trimethylamine precursors or when there is bacterial overgrowth in the bowel resulting in increased production of trimethylamine. […] The enzyme may be underactive in liver and kidney disease, during menstruation or in the presence of inhibitors such as those derived from eating Brussel sprouts, oral thiourea or topical hydroquinone. […] It is important to distinguish this form from primary trimethylaminuria by measuring both the trimethylamine and trimethylamine N-oxide levels in urine.

• #11 Trimethylaminuria – Wikipedia

  https://en.wikipedia.org/wiki/Trimethylaminuria

  Normally, the FMO3 enzyme converts fishy-smelling trimethylamine into trimethylamine N-oxide which has no odor. If the enzyme is missing or its activity is reduced because of a mutation in the FMO3 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. […] There are more than 40 known mutations associated with TMAU. Loss-of-function mutations, nonsense mutations, and missense mutations are three of the most common. Nonsense and missense mutations cause the most severe phenotypes. […] 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.

• #12 Trimethylaminuria – Wikipedia

  https://en.wikipedia.org/wiki/Trimethylaminuria

  Trimethylaminuria (TMAU), also known as fish odor syndrome or fish malodor syndrome, is a rare metabolic disorder that causes a defect in the normal production of an enzyme named flavin-containing monooxygenase 3 (FMO3). When FMO3 is not working correctly or if not enough enzyme is produced, the body loses the ability to properly convert the fishy-smelling chemical trimethylamine (TMA) from precursor compounds in food digestion into trimethylamine oxide (TMAO), through a process called N-oxidation. […] Trimethylamine then builds up and is released in the person’s sweat, urine, and breath, giving off a fishy odor. Primary trimethylaminuria is caused by genetic mutations that affect the FMO3 function of the liver. […] Mutations in the FMO3 gene, which is found on the long arm of chromosome 1, cause trimethylaminuria. The FMO3 gene makes an enzyme that breaks down nitrogen-containing compounds from the diet, including trimethylamine.

• #13 A Review of Trimethylaminuria: (Fish Odor Syndrome)

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3848652/

  Trimethylaminuria, better known as fish odor syndrome, is a psychologically disabling condition in which a patient emits a foul odor, which resembles that of rotting fish. The disorder is most commonly caused by an inherited deficiency in flavin monooxygenase 3, the vital enzyme for the metabolism of trimethylamine, which is the compound responsible for the unpleasant odor. […] The cause of the syndrome is rooted in the dysfunctional metabolism of TMA, which is normally oxidized by flavin monooxygenase 3 (FMO3) into non-odorous trimethylamine-N-oxide (TMAO). […] Most patients with FOS are eventually diagnosed with primary trimethylaminuria, which is caused by a deficiency in FMO3 that is inherited in an autosomal recessive fashion. Under normal circumstances, dietary TMAO and other TMA precursors, such as choline, are ingested and then reduced to TMA by colonic bacteria.

• #14 Trimethylaminuria (Fish Odor Syndrome or TMAU)

  https://my.clevelandclinic.org/health/diseases/22356-trimethylaminuria-fish-odor-syndrome

  Trimethylaminuria (TMAU, fish odor syndrome) is an uncommon condition that makes people smell like rotten fish. Its a metabolic disorder. […] Trimethylaminuria happens when something affects the FMO3 enzyme so it doesnt work like it should. FMO3 stands for flavin-containing monooxygenase 3. This enzyme manages the impact that trimethylamine has on your body. Trimethylamine is a smelly chemical your body produces when you eat certain foods like fish, beans and eggs. […] Normally, theres a check-and-balance relationship between trimethylamine and FMO3: when your body produces trimethylamine, FMO3 enzymes break it down. That way, the chemical doesnt smell bad when it moves from your digestive system into your bloodstream. When that doesnt happen, trimethylamine builds up in your body, eventually seeping into your system so that your breath, sweat, saliva and pee smell like rotten fish.

• #15 Trimethylaminuria

  https://dermnetnz.org/topics/trimethylaminuria

  Trimethylaminuria is also known as fish (mal)odour syndrome because of the characteristic fishy body odour. […] Trimethylamine is a volatile aliphatic molecule, best known as the smell of rotting fish. […] Bacteria in the bowel produce the malodourous trimethylamine from trimethylamine N-oxide or choline. […] Normally it is converted to the odourless trimethylamine N-oxide by an enzyme in the liver, known as trimethylamine oxidase or flavin-containing mono-oxygenase 3 (FMO3). […] In trimethylaminuria, this malodourous molecule is excreted in sweat, urine, breath, saliva, vaginal and other body secretions. […] Primary trimethylaminuria is a rare autosomal recessive genetic disease (MIM 602079), meaning the affected person has inherited two copies of the defective gene, one from each parent.

• #16 Trimethylaminuria – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK594255/

  Trimethylaminuria (TMAU) is a rare metabolic disorder resulting in the accumulation of trimethylamine. Primary TMAU is an autosomal recessive genetic condition that results in the deficiency or dysfunction of the hepatic enzyme flavin monooxygenase 3. The underlying pathogenesis of TMAU is usually a deficient or dysfunctional hepatic enzyme, flavin-containing monooxygenase 3 (FMO3). This enzyme metabolizes TMA into an odorless compound, trimethylamine N-oxide (TMAO. Primary trimethylaminuria occurs secondary to a genetic mutation of the FMO3 gene, located on chromosome 1q24.3. Various FMO3 mutations result in decreased enzyme activity, impaired substrate binding, or disrupted protein structure. Failure to metabolize TMA results in its accumulation; it is subsequently excreted via urine, sweat, breath, and other body secretions. The excess excretion is what causes the fishy odor. Primary TMAU is caused by a dysfunction or deficiency of the flavin-containing monooxygenase 3 (FMO3) enzyme due to mutations in FMO3. The FMO3 enzyme plays a crucial role in the metabolism of TMA, a compound with a fishy odor. The dysfunctional or deficient FMO3 enzyme in patients with primary TMAU impairs TMA conversion, resulting in an increased TMA:TMAO ratio. The accumulated TMA is passively absorbed into the bloodstream and is released through various excretory routes, including sweat, breath, and urine, giving rise to the distinctive fishy odor associated with TMAU. The gut microbiota produces TMA by metabolizing certain nitrogen-containing compounds, such as choline and carnitine, in certain foods. In individuals with TMAU, the excessive production of TMA by colonic bacteria further contributes to the accumulation of TMA in the body.

• #17 Trimethylaminuria – Metabolic Support UKAccessibility ToolsIncrease TextDecrease TextGrayscaleHigh ContrastNegative ContrastLight BackgroundLinks UnderlineReadable FontReset

  https://metabolicsupportuk.org/condition/trimethylaminuria/

  Trimethylaminuria is caused by a problem in the FMO3 gene. This gene produces the FMO3 enzyme, which breaks down a compound called trimethylamine that is produced in your gut when you eat certain protein-rich food. This process is needed in order to reduce the amount of trimethylamine in the body and to convert it into an odourless molecule. […] If you have a problem in the FMO3 gene, you will not produce enough of the FMO3 enzyme. Therefore, you will be unable to break down trimethylamine, and this causes it to build up until it is released as a strong, unpleasant odour through your sweat, breath, and urine.

• #18 Trimethylaminuria – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK594255/

  Trimethylaminuria (TMAU) is a rare metabolic disorder resulting in the accumulation of trimethylamine. Primary TMAU is an autosomal recessive genetic condition that results in the deficiency or dysfunction of the hepatic enzyme flavin monooxygenase 3. The underlying pathogenesis of TMAU is usually a deficient or dysfunctional hepatic enzyme, flavin-containing monooxygenase 3 (FMO3). This enzyme metabolizes TMA into an odorless compound, trimethylamine N-oxide (TMAO. Primary trimethylaminuria occurs secondary to a genetic mutation of the FMO3 gene, located on chromosome 1q24.3. Various FMO3 mutations result in decreased enzyme activity, impaired substrate binding, or disrupted protein structure. Failure to metabolize TMA results in its accumulation; it is subsequently excreted via urine, sweat, breath, and other body secretions. The excess excretion is what causes the fishy odor. Primary TMAU is caused by a dysfunction or deficiency of the flavin-containing monooxygenase 3 (FMO3) enzyme due to mutations in FMO3. The FMO3 enzyme plays a crucial role in the metabolism of TMA, a compound with a fishy odor. The dysfunctional or deficient FMO3 enzyme in patients with primary TMAU impairs TMA conversion, resulting in an increased TMA:TMAO ratio. The accumulated TMA is passively absorbed into the bloodstream and is released through various excretory routes, including sweat, breath, and urine, giving rise to the distinctive fishy odor associated with TMAU. The gut microbiota produces TMA by metabolizing certain nitrogen-containing compounds, such as choline and carnitine, in certain foods. In individuals with TMAU, the excessive production of TMA by colonic bacteria further contributes to the accumulation of TMA in the body.

• #19 A Review of Trimethylaminuria: (Fish Odor Syndrome)

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3848652/

  Trimethylaminuria, better known as fish odor syndrome, is a psychologically disabling condition in which a patient emits a foul odor, which resembles that of rotting fish. The disorder is most commonly caused by an inherited deficiency in flavin monooxygenase 3, the vital enzyme for the metabolism of trimethylamine, which is the compound responsible for the unpleasant odor. […] The cause of the syndrome is rooted in the dysfunctional metabolism of TMA, which is normally oxidized by flavin monooxygenase 3 (FMO3) into non-odorous trimethylamine-N-oxide (TMAO). […] Most patients with FOS are eventually diagnosed with primary trimethylaminuria, which is caused by a deficiency in FMO3 that is inherited in an autosomal recessive fashion. Under normal circumstances, dietary TMAO and other TMA precursors, such as choline, are ingested and then reduced to TMA by colonic bacteria.

• #20 Trimethylaminuria – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK594255/

  Trimethylaminuria (TMAU) is a rare metabolic disorder resulting in the accumulation of trimethylamine. Primary TMAU is an autosomal recessive genetic condition that results in the deficiency or dysfunction of the hepatic enzyme flavin monooxygenase 3. The underlying pathogenesis of TMAU is usually a deficient or dysfunctional hepatic enzyme, flavin-containing monooxygenase 3 (FMO3). This enzyme metabolizes TMA into an odorless compound, trimethylamine N-oxide (TMAO. Primary trimethylaminuria occurs secondary to a genetic mutation of the FMO3 gene, located on chromosome 1q24.3. Various FMO3 mutations result in decreased enzyme activity, impaired substrate binding, or disrupted protein structure. Failure to metabolize TMA results in its accumulation; it is subsequently excreted via urine, sweat, breath, and other body secretions. The excess excretion is what causes the fishy odor. Primary TMAU is caused by a dysfunction or deficiency of the flavin-containing monooxygenase 3 (FMO3) enzyme due to mutations in FMO3. The FMO3 enzyme plays a crucial role in the metabolism of TMA, a compound with a fishy odor. The dysfunctional or deficient FMO3 enzyme in patients with primary TMAU impairs TMA conversion, resulting in an increased TMA:TMAO ratio. The accumulated TMA is passively absorbed into the bloodstream and is released through various excretory routes, including sweat, breath, and urine, giving rise to the distinctive fishy odor associated with TMAU. The gut microbiota produces TMA by metabolizing certain nitrogen-containing compounds, such as choline and carnitine, in certain foods. In individuals with TMAU, the excessive production of TMA by colonic bacteria further contributes to the accumulation of TMA in the body.

• #21 Trimethylaminuria

  https://dermnetnz.org/topics/trimethylaminuria

  Trimethylaminuria is also known as fish (mal)odour syndrome because of the characteristic fishy body odour. […] Trimethylamine is a volatile aliphatic molecule, best known as the smell of rotting fish. […] Bacteria in the bowel produce the malodourous trimethylamine from trimethylamine N-oxide or choline. […] Normally it is converted to the odourless trimethylamine N-oxide by an enzyme in the liver, known as trimethylamine oxidase or flavin-containing mono-oxygenase 3 (FMO3). […] In trimethylaminuria, this malodourous molecule is excreted in sweat, urine, breath, saliva, vaginal and other body secretions. […] Primary trimethylaminuria is a rare autosomal recessive genetic disease (MIM 602079), meaning the affected person has inherited two copies of the defective gene, one from each parent.

• #22 A Review of Trimethylaminuria: (Fish Odor Syndrome)

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3848652/

  While primary trimethylaminuria accounts for the majority of cases, there also exists secondary or acquired trimethylaminuria where FMO3 may still retain varying degrees of functionality. […] Regardless of the etiology, FOS is often associated with significant psychosocial disturbances, which must be considered when developing a treatment plan. […] The diagnosis, which often eludes practitioners for years, is made on the basis of the clinical presentation and urinalysis. […] Once the diagnosis has been established, there are a number of potentially helpful treatment options; however, no single regimen seems universally efficacious. […] Another logical solution to FOS is to exclude or reduce TMAO, a TMA precursor, from the diet, which is found in high concentration in marine fish. […] Yamazaki et al studied the ability of sequestering agents to improve FOS as determined by the oxidizing ratio in the urine.

• #23 A Review of Trimethylaminuria (Fish Odor Syndrome) | JCAD – The Journal of Clinical and Aesthetic Dermatology

  https://jcadonline.com/a-review-of-trimethylaminuria-fish-odor-syndrome/

  While primary trimethylaminuria accounts for the majority of cases, there also exists secondary or acquired trimethylaminuria where FMO3 may still retain varying degrees of functionality. […] The mechanism involves the destruction of gut bacteria, which are responsible for the reduction of TMAO into TMA. However, antimicrobial therapy only moderately alleviated the foul odor and presents a number of possible side effects if used chronically, so this modality is best suited for transient cases or in instances where bacterial overgrowth is thought to be a major factor in the pathogenesis of FOS. […] TMAU is most commonly an inherited disorder that is characterized by an unpleasant odor similar to that of rotting fish. The disease was once considered quite rare, but recent research has demonstrated that the condition may be more prevalent than historically appreciated.

• #24 Trimethylaminuria

  https://dermnetnz.org/topics/trimethylaminuria

  Two defective copies of the gene result in a failure to produce sufficient active FMO3 enzyme. […] Some mutations cause a reduction in FMO3 enzyme activity and others result in complete loss of enzyme activity. […] Secondary trimethylaminuria occurs when the liver FMO3 enzyme is either overwhelmed or underactive for some reason. […] The enzyme may be overwhelmed by an excessive dietary intake of trimethylamine precursors or when there is bacterial overgrowth in the bowel resulting in increased production of trimethylamine. […] The enzyme may be underactive in liver and kidney disease, during menstruation or in the presence of inhibitors such as those derived from eating Brussel sprouts, oral thiourea or topical hydroquinone. […] It is important to distinguish this form from primary trimethylaminuria by measuring both the trimethylamine and trimethylamine N-oxide levels in urine.

• #25 Trimethylaminuria

  https://dermnetnz.org/topics/trimethylaminuria

  Two defective copies of the gene result in a failure to produce sufficient active FMO3 enzyme. […] Some mutations cause a reduction in FMO3 enzyme activity and others result in complete loss of enzyme activity. […] Secondary trimethylaminuria occurs when the liver FMO3 enzyme is either overwhelmed or underactive for some reason. […] The enzyme may be overwhelmed by an excessive dietary intake of trimethylamine precursors or when there is bacterial overgrowth in the bowel resulting in increased production of trimethylamine. […] The enzyme may be underactive in liver and kidney disease, during menstruation or in the presence of inhibitors such as those derived from eating Brussel sprouts, oral thiourea or topical hydroquinone. […] It is important to distinguish this form from primary trimethylaminuria by measuring both the trimethylamine and trimethylamine N-oxide levels in urine.

• #26 Trimethylaminuria

  https://dermnetnz.org/topics/trimethylaminuria

  Two defective copies of the gene result in a failure to produce sufficient active FMO3 enzyme. […] Some mutations cause a reduction in FMO3 enzyme activity and others result in complete loss of enzyme activity. […] Secondary trimethylaminuria occurs when the liver FMO3 enzyme is either overwhelmed or underactive for some reason. […] The enzyme may be overwhelmed by an excessive dietary intake of trimethylamine precursors or when there is bacterial overgrowth in the bowel resulting in increased production of trimethylamine. […] The enzyme may be underactive in liver and kidney disease, during menstruation or in the presence of inhibitors such as those derived from eating Brussel sprouts, oral thiourea or topical hydroquinone. […] It is important to distinguish this form from primary trimethylaminuria by measuring both the trimethylamine and trimethylamine N-oxide levels in urine.

• #27 Trimethylaminuria

  https://dermnetnz.org/topics/trimethylaminuria

  Two defective copies of the gene result in a failure to produce sufficient active FMO3 enzyme. […] Some mutations cause a reduction in FMO3 enzyme activity and others result in complete loss of enzyme activity. […] Secondary trimethylaminuria occurs when the liver FMO3 enzyme is either overwhelmed or underactive for some reason. […] The enzyme may be overwhelmed by an excessive dietary intake of trimethylamine precursors or when there is bacterial overgrowth in the bowel resulting in increased production of trimethylamine. […] The enzyme may be underactive in liver and kidney disease, during menstruation or in the presence of inhibitors such as those derived from eating Brussel sprouts, oral thiourea or topical hydroquinone. […] It is important to distinguish this form from primary trimethylaminuria by measuring both the trimethylamine and trimethylamine N-oxide levels in urine.

• #28 Trimethylaminuria

  https://dermnetnz.org/topics/trimethylaminuria

  Two defective copies of the gene result in a failure to produce sufficient active FMO3 enzyme. […] Some mutations cause a reduction in FMO3 enzyme activity and others result in complete loss of enzyme activity. […] Secondary trimethylaminuria occurs when the liver FMO3 enzyme is either overwhelmed or underactive for some reason. […] The enzyme may be overwhelmed by an excessive dietary intake of trimethylamine precursors or when there is bacterial overgrowth in the bowel resulting in increased production of trimethylamine. […] The enzyme may be underactive in liver and kidney disease, during menstruation or in the presence of inhibitors such as those derived from eating Brussel sprouts, oral thiourea or topical hydroquinone. […] It is important to distinguish this form from primary trimethylaminuria by measuring both the trimethylamine and trimethylamine N-oxide levels in urine.

• #29 Trimethylaminuria

  https://dermnetnz.org/topics/trimethylaminuria

  Two defective copies of the gene result in a failure to produce sufficient active FMO3 enzyme. […] Some mutations cause a reduction in FMO3 enzyme activity and others result in complete loss of enzyme activity. […] Secondary trimethylaminuria occurs when the liver FMO3 enzyme is either overwhelmed or underactive for some reason. […] The enzyme may be overwhelmed by an excessive dietary intake of trimethylamine precursors or when there is bacterial overgrowth in the bowel resulting in increased production of trimethylamine. […] The enzyme may be underactive in liver and kidney disease, during menstruation or in the presence of inhibitors such as those derived from eating Brussel sprouts, oral thiourea or topical hydroquinone. […] It is important to distinguish this form from primary trimethylaminuria by measuring both the trimethylamine and trimethylamine N-oxide levels in urine.

• #30 Trimethylaminuria

  https://dermnetnz.org/topics/trimethylaminuria

  Two defective copies of the gene result in a failure to produce sufficient active FMO3 enzyme. […] Some mutations cause a reduction in FMO3 enzyme activity and others result in complete loss of enzyme activity. […] Secondary trimethylaminuria occurs when the liver FMO3 enzyme is either overwhelmed or underactive for some reason. […] The enzyme may be overwhelmed by an excessive dietary intake of trimethylamine precursors or when there is bacterial overgrowth in the bowel resulting in increased production of trimethylamine. […] The enzyme may be underactive in liver and kidney disease, during menstruation or in the presence of inhibitors such as those derived from eating Brussel sprouts, oral thiourea or topical hydroquinone. […] It is important to distinguish this form from primary trimethylaminuria by measuring both the trimethylamine and trimethylamine N-oxide levels in urine.

• #31 Trimethylaminuria – Wikipedia

  https://en.wikipedia.org/wiki/Trimethylaminuria

  Normally, the FMO3 enzyme converts fishy-smelling trimethylamine into trimethylamine N-oxide which has no odor. If the enzyme is missing or its activity is reduced because of a mutation in the FMO3 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. […] There are more than 40 known mutations associated with TMAU. Loss-of-function mutations, nonsense mutations, and missense mutations are three of the most common. Nonsense and missense mutations cause the most severe phenotypes. […] 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.

• #32 Trimethylaminuria

  https://dermnetnz.org/topics/trimethylaminuria

  The diagnosis should be considered in patients presenting because of body odour, especially if described as fishy. […] The diagnosis is confirmed on 24-hour urine collection while on a normal diet, and an 8-hour urine collection after either a marine fish meal (for children) or 600mg oral trimethylamine load (adults). […] Both trimethylamine and trimethylamine N-oxide should be measured. […] Dietary modification is the basis of treatment as avoidance of trimethylamine precursors reduces the body odour. […] Marine (sea- or salt-water) fish, including cephalopods and crustaceans, must be avoided completely as they have the highest concentration of the precursor trimethylamine N-oxide. […] Some sufferers respond well to courses of neomycin, amoxicillin or metronidazole as these alter the bowel bacteria, reducing the production of trimethylamine. […] Hydroquinone used topically as a depigmenting agent has been reported to trigger the fish odour in those using the drug in large amount for a long time.

• #33 Trimethylaminuria (Fish Odor Syndrome or TMAU)

  https://my.clevelandclinic.org/health/diseases/22356-trimethylaminuria-fish-odor-syndrome

  Trimethylaminuria (TMAU, fish odor syndrome) is an uncommon condition that makes people smell like rotten fish. Its a metabolic disorder. […] Trimethylaminuria happens when something affects the FMO3 enzyme so it doesnt work like it should. FMO3 stands for flavin-containing monooxygenase 3. This enzyme manages the impact that trimethylamine has on your body. Trimethylamine is a smelly chemical your body produces when you eat certain foods like fish, beans and eggs. […] Normally, theres a check-and-balance relationship between trimethylamine and FMO3: when your body produces trimethylamine, FMO3 enzymes break it down. That way, the chemical doesnt smell bad when it moves from your digestive system into your bloodstream. When that doesnt happen, trimethylamine builds up in your body, eventually seeping into your system so that your breath, sweat, saliva and pee smell like rotten fish.

• #34 Trimethylaminuria – Wikipedia

  https://en.wikipedia.org/wiki/Trimethylaminuria

  Normally, the FMO3 enzyme converts fishy-smelling trimethylamine into trimethylamine N-oxide which has no odor. If the enzyme is missing or its activity is reduced because of a mutation in the FMO3 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. […] There are more than 40 known mutations associated with TMAU. Loss-of-function mutations, nonsense mutations, and missense mutations are three of the most common. Nonsense and missense mutations cause the most severe phenotypes. […] 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.

• #35 A Review of Trimethylaminuria (Fish Odor Syndrome) | JCAD – The Journal of Clinical and Aesthetic Dermatology

  https://jcadonline.com/a-review-of-trimethylaminuria-fish-odor-syndrome/

  While primary trimethylaminuria accounts for the majority of cases, there also exists secondary or acquired trimethylaminuria where FMO3 may still retain varying degrees of functionality. […] The mechanism involves the destruction of gut bacteria, which are responsible for the reduction of TMAO into TMA. However, antimicrobial therapy only moderately alleviated the foul odor and presents a number of possible side effects if used chronically, so this modality is best suited for transient cases or in instances where bacterial overgrowth is thought to be a major factor in the pathogenesis of FOS. […] TMAU is most commonly an inherited disorder that is characterized by an unpleasant odor similar to that of rotting fish. The disease was once considered quite rare, but recent research has demonstrated that the condition may be more prevalent than historically appreciated.

• #36 Trimethylaminuria

  https://dermnetnz.org/topics/trimethylaminuria

  Two defective copies of the gene result in a failure to produce sufficient active FMO3 enzyme. […] Some mutations cause a reduction in FMO3 enzyme activity and others result in complete loss of enzyme activity. […] Secondary trimethylaminuria occurs when the liver FMO3 enzyme is either overwhelmed or underactive for some reason. […] The enzyme may be overwhelmed by an excessive dietary intake of trimethylamine precursors or when there is bacterial overgrowth in the bowel resulting in increased production of trimethylamine. […] The enzyme may be underactive in liver and kidney disease, during menstruation or in the presence of inhibitors such as those derived from eating Brussel sprouts, oral thiourea or topical hydroquinone. […] It is important to distinguish this form from primary trimethylaminuria by measuring both the trimethylamine and trimethylamine N-oxide levels in urine.

• #37 A Review of Trimethylaminuria (Fish Odor Syndrome) | JCAD – The Journal of Clinical and Aesthetic Dermatology

  https://jcadonline.com/a-review-of-trimethylaminuria-fish-odor-syndrome/

  While primary trimethylaminuria accounts for the majority of cases, there also exists secondary or acquired trimethylaminuria where FMO3 may still retain varying degrees of functionality. […] The mechanism involves the destruction of gut bacteria, which are responsible for the reduction of TMAO into TMA. However, antimicrobial therapy only moderately alleviated the foul odor and presents a number of possible side effects if used chronically, so this modality is best suited for transient cases or in instances where bacterial overgrowth is thought to be a major factor in the pathogenesis of FOS. […] TMAU is most commonly an inherited disorder that is characterized by an unpleasant odor similar to that of rotting fish. The disease was once considered quite rare, but recent research has demonstrated that the condition may be more prevalent than historically appreciated.

• #38

  https://111.wales.nhs.uk/encyclopaedia/t/article/Trimethylaminuria(’fishodoursyndrome’)/

  Trimethylaminuria (TMAU) is an uncommon condition that causes an unpleasant, fishy smell. It’s also called „fish odour syndrome.” […] In trimethylaminuria, the body is unable to turn a strong-smelling chemical called trimethylamine produced in the gut when bacteria break down certain foods into a different chemical that doesn’t smell. […] This means trimethylamine builds up in the body and gets into bodily fluids like sweat. […] Many people with trimethylaminuria inherit a faulty version of a gene called FMO3 from both their parents. This means they have 2 copies of the faulty gene. […] There’s currently no cure for trimethylaminuria, but some things might help with the smell. […] Your doctor may recommend short courses of antibiotics this can help reduce the amount of trimethylamine produced in your gut.

• #39 A Review of Trimethylaminuria (Fish Odor Syndrome) | JCAD – The Journal of Clinical and Aesthetic Dermatology

  https://jcadonline.com/a-review-of-trimethylaminuria-fish-odor-syndrome/

  While primary trimethylaminuria accounts for the majority of cases, there also exists secondary or acquired trimethylaminuria where FMO3 may still retain varying degrees of functionality. […] The mechanism involves the destruction of gut bacteria, which are responsible for the reduction of TMAO into TMA. However, antimicrobial therapy only moderately alleviated the foul odor and presents a number of possible side effects if used chronically, so this modality is best suited for transient cases or in instances where bacterial overgrowth is thought to be a major factor in the pathogenesis of FOS. […] TMAU is most commonly an inherited disorder that is characterized by an unpleasant odor similar to that of rotting fish. The disease was once considered quite rare, but recent research has demonstrated that the condition may be more prevalent than historically appreciated.

• #40 Trimethylaminuria

  https://dermnetnz.org/topics/trimethylaminuria

  Trimethylaminuria is also known as fish (mal)odour syndrome because of the characteristic fishy body odour. […] Trimethylamine is a volatile aliphatic molecule, best known as the smell of rotting fish. […] Bacteria in the bowel produce the malodourous trimethylamine from trimethylamine N-oxide or choline. […] Normally it is converted to the odourless trimethylamine N-oxide by an enzyme in the liver, known as trimethylamine oxidase or flavin-containing mono-oxygenase 3 (FMO3). […] In trimethylaminuria, this malodourous molecule is excreted in sweat, urine, breath, saliva, vaginal and other body secretions. […] Primary trimethylaminuria is a rare autosomal recessive genetic disease (MIM 602079), meaning the affected person has inherited two copies of the defective gene, one from each parent.

• #41 Trimethylaminuria – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK594255/

  Trimethylaminuria (TMAU) is a rare metabolic disorder resulting in the accumulation of trimethylamine. Primary TMAU is an autosomal recessive genetic condition that results in the deficiency or dysfunction of the hepatic enzyme flavin monooxygenase 3. The underlying pathogenesis of TMAU is usually a deficient or dysfunctional hepatic enzyme, flavin-containing monooxygenase 3 (FMO3). This enzyme metabolizes TMA into an odorless compound, trimethylamine N-oxide (TMAO. Primary trimethylaminuria occurs secondary to a genetic mutation of the FMO3 gene, located on chromosome 1q24.3. Various FMO3 mutations result in decreased enzyme activity, impaired substrate binding, or disrupted protein structure. Failure to metabolize TMA results in its accumulation; it is subsequently excreted via urine, sweat, breath, and other body secretions. The excess excretion is what causes the fishy odor. Primary TMAU is caused by a dysfunction or deficiency of the flavin-containing monooxygenase 3 (FMO3) enzyme due to mutations in FMO3. The FMO3 enzyme plays a crucial role in the metabolism of TMA, a compound with a fishy odor. The dysfunctional or deficient FMO3 enzyme in patients with primary TMAU impairs TMA conversion, resulting in an increased TMA:TMAO ratio. The accumulated TMA is passively absorbed into the bloodstream and is released through various excretory routes, including sweat, breath, and urine, giving rise to the distinctive fishy odor associated with TMAU. The gut microbiota produces TMA by metabolizing certain nitrogen-containing compounds, such as choline and carnitine, in certain foods. In individuals with TMAU, the excessive production of TMA by colonic bacteria further contributes to the accumulation of TMA in the body.

• #42 Trimethylaminuria

  https://dermnetnz.org/topics/trimethylaminuria

  Trimethylaminuria is also known as fish (mal)odour syndrome because of the characteristic fishy body odour. […] Trimethylamine is a volatile aliphatic molecule, best known as the smell of rotting fish. […] Bacteria in the bowel produce the malodourous trimethylamine from trimethylamine N-oxide or choline. […] Normally it is converted to the odourless trimethylamine N-oxide by an enzyme in the liver, known as trimethylamine oxidase or flavin-containing mono-oxygenase 3 (FMO3). […] In trimethylaminuria, this malodourous molecule is excreted in sweat, urine, breath, saliva, vaginal and other body secretions. […] Primary trimethylaminuria is a rare autosomal recessive genetic disease (MIM 602079), meaning the affected person has inherited two copies of the defective gene, one from each parent.

• #43 Trimethylaminuria (Fish Odor Syndrome or TMAU)

  https://my.clevelandclinic.org/health/diseases/22356-trimethylaminuria-fish-odor-syndrome

  Trimethylaminuria (TMAU, fish odor syndrome) is an uncommon condition that makes people smell like rotten fish. Its a metabolic disorder. […] Trimethylaminuria happens when something affects the FMO3 enzyme so it doesnt work like it should. FMO3 stands for flavin-containing monooxygenase 3. This enzyme manages the impact that trimethylamine has on your body. Trimethylamine is a smelly chemical your body produces when you eat certain foods like fish, beans and eggs. […] Normally, theres a check-and-balance relationship between trimethylamine and FMO3: when your body produces trimethylamine, FMO3 enzymes break it down. That way, the chemical doesnt smell bad when it moves from your digestive system into your bloodstream. When that doesnt happen, trimethylamine builds up in your body, eventually seeping into your system so that your breath, sweat, saliva and pee smell like rotten fish.

• #44

  https://111.wales.nhs.uk/encyclopaedia/t/article/Trimethylaminuria(’fishodoursyndrome’)/

  Trimethylaminuria (TMAU) is an uncommon condition that causes an unpleasant, fishy smell. It’s also called „fish odour syndrome.” […] In trimethylaminuria, the body is unable to turn a strong-smelling chemical called trimethylamine produced in the gut when bacteria break down certain foods into a different chemical that doesn’t smell. […] This means trimethylamine builds up in the body and gets into bodily fluids like sweat. […] Many people with trimethylaminuria inherit a faulty version of a gene called FMO3 from both their parents. This means they have 2 copies of the faulty gene. […] There’s currently no cure for trimethylaminuria, but some things might help with the smell. […] Your doctor may recommend short courses of antibiotics this can help reduce the amount of trimethylamine produced in your gut.

• #45 Trimethylaminuria – Metabolic Support UKAccessibility ToolsIncrease TextDecrease TextGrayscaleHigh ContrastNegative ContrastLight BackgroundLinks UnderlineReadable FontReset

  https://metabolicsupportuk.org/condition/trimethylaminuria/

  Trimethylaminuria is caused by a problem in the FMO3 gene. This gene produces the FMO3 enzyme, which breaks down a compound called trimethylamine that is produced in your gut when you eat certain protein-rich food. This process is needed in order to reduce the amount of trimethylamine in the body and to convert it into an odourless molecule. […] If you have a problem in the FMO3 gene, you will not produce enough of the FMO3 enzyme. Therefore, you will be unable to break down trimethylamine, and this causes it to build up until it is released as a strong, unpleasant odour through your sweat, breath, and urine.

• #46 A Review of Trimethylaminuria (Fish Odor Syndrome) | JCAD – The Journal of Clinical and Aesthetic Dermatology

  https://jcadonline.com/a-review-of-trimethylaminuria-fish-odor-syndrome/

  Trimethylaminuria, better known as fish odor syndrome, is a psychologically disabling condition in which a patient emits a foul odor, which resembles that of rotting fish. The disorder is most commonly caused by an inherited deficiency in flavin monooxygenase 3, the vital enzyme for the metabolism of trimethylamine, which is the compound responsible for the unpleasant odor. […] The cause of the syndrome is rooted in the dysfunctional metabolism of TMA, which is normally oxidized by flavin monooxygenase 3 (FMO3) into non-odorous trimethylamine-N-oxide (TMAO). […] Most patients with FOS are eventually diagnosed with primary trimethylaminuria, which is caused by a deficiency in FMO3 that is inherited in an autosomal recessive fashion. Under normal circumstances, dietary TMAO and other TMA precursors, such as choline, are ingested and then reduced to TMA by colonic bacteria.

• #47 Trimethylaminuria – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK594255/

  Trimethylaminuria (TMAU) is a rare metabolic disorder resulting in the accumulation of trimethylamine. Primary TMAU is an autosomal recessive genetic condition that results in the deficiency or dysfunction of the hepatic enzyme flavin monooxygenase 3. The underlying pathogenesis of TMAU is usually a deficient or dysfunctional hepatic enzyme, flavin-containing monooxygenase 3 (FMO3). This enzyme metabolizes TMA into an odorless compound, trimethylamine N-oxide (TMAO. Primary trimethylaminuria occurs secondary to a genetic mutation of the FMO3 gene, located on chromosome 1q24.3. Various FMO3 mutations result in decreased enzyme activity, impaired substrate binding, or disrupted protein structure. Failure to metabolize TMA results in its accumulation; it is subsequently excreted via urine, sweat, breath, and other body secretions. The excess excretion is what causes the fishy odor. Primary TMAU is caused by a dysfunction or deficiency of the flavin-containing monooxygenase 3 (FMO3) enzyme due to mutations in FMO3. The FMO3 enzyme plays a crucial role in the metabolism of TMA, a compound with a fishy odor. The dysfunctional or deficient FMO3 enzyme in patients with primary TMAU impairs TMA conversion, resulting in an increased TMA:TMAO ratio. The accumulated TMA is passively absorbed into the bloodstream and is released through various excretory routes, including sweat, breath, and urine, giving rise to the distinctive fishy odor associated with TMAU. The gut microbiota produces TMA by metabolizing certain nitrogen-containing compounds, such as choline and carnitine, in certain foods. In individuals with TMAU, the excessive production of TMA by colonic bacteria further contributes to the accumulation of TMA in the body.

• #48 Trimethylaminuria – Wikipedia

  https://en.wikipedia.org/wiki/Trimethylaminuria

  Trimethylaminuria (TMAU), also known as fish odor syndrome or fish malodor syndrome, is a rare metabolic disorder that causes a defect in the normal production of an enzyme named flavin-containing monooxygenase 3 (FMO3). When FMO3 is not working correctly or if not enough enzyme is produced, the body loses the ability to properly convert the fishy-smelling chemical trimethylamine (TMA) from precursor compounds in food digestion into trimethylamine oxide (TMAO), through a process called N-oxidation. […] Trimethylamine then builds up and is released in the person’s sweat, urine, and breath, giving off a fishy odor. Primary trimethylaminuria is caused by genetic mutations that affect the FMO3 function of the liver. […] Mutations in the FMO3 gene, which is found on the long arm of chromosome 1, cause trimethylaminuria. The FMO3 gene makes an enzyme that breaks down nitrogen-containing compounds from the diet, including trimethylamine.

• #49 A Review of Trimethylaminuria: (Fish Odor Syndrome)

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3848652/

  While primary trimethylaminuria accounts for the majority of cases, there also exists secondary or acquired trimethylaminuria where FMO3 may still retain varying degrees of functionality. […] Regardless of the etiology, FOS is often associated with significant psychosocial disturbances, which must be considered when developing a treatment plan. […] The diagnosis, which often eludes practitioners for years, is made on the basis of the clinical presentation and urinalysis. […] Once the diagnosis has been established, there are a number of potentially helpful treatment options; however, no single regimen seems universally efficacious. […] Another logical solution to FOS is to exclude or reduce TMAO, a TMA precursor, from the diet, which is found in high concentration in marine fish. […] Yamazaki et al studied the ability of sequestering agents to improve FOS as determined by the oxidizing ratio in the urine.

• #50 A Review of Trimethylaminuria: (Fish Odor Syndrome)

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3848652/

  The mechanism involves the destruction of gut bacteria, which are responsible for the reduction of TMAO into TMA. […] TMAU is most commonly an inherited disorder that is characterized by an unpleasant odor similar to that of rotting fish. The disease itself is not life threatening, but it significantly diminishes the quality of patients lives, and has even been associated with suicide attempts. […] There are many options to reduce the severity of the condition, which range from simple lifestyle changes, such as washing with acidic soap and eating a choline-deficient diet to utilizing antibiotics and sequestering agents.

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