TMAU
MCID: TRM004
MIFTS: 49

Trimethylaminuria (TMAU)

Categories: Genetic diseases, Metabolic diseases, Rare diseases

Aliases & Classifications for Trimethylaminuria

MalaCards integrated aliases for Trimethylaminuria:

Name: Trimethylaminuria 57 12 75 53 25 74 37 29 13 55 6 44 15 72
Tmau 57 53 25 74
Fish-Odor Syndrome 57 12 74
Fish Malodor Syndrome 53 25
Stale Fish Syndrome 53 25
Fish Odor Syndrome 53 25
Tmauria 53 25
Severe Primary Trimethylaminuria 59
Trimethylaminuria ) 40
Mesh; D008661 74

Characteristics:

OMIM:

57
Inheritance:
autosomal recessive

Miscellaneous:
offensive fishy body odor


HPO:

32
trimethylaminuria:
Inheritance autosomal recessive inheritance


Classifications:

Orphanet: 59  
Inborn errors of metabolism


External Ids:

Disease Ontology 12 DOID:0080361
OMIM 57 602079
KEGG 37 H01234
MeSH 44 C536561
ICD10 via Orphanet 34 E88.8
Orphanet 59 ORPHA468726
MedGen 42 C0342739
UMLS 72 C0342739

Summaries for Trimethylaminuria

NIH Rare Diseases : 53 Trimethylaminuria is a metabolic disorder that occurs when the body is unable to break down certain nitrogen-containing compounds such as trimethylamine. Trimethylamine, which has a fishy odor, is produced in the intestines when certain types of food (i.e. eggs, liver, legumes, fish and some vegetables) are digested. As trimethylamine begins to accumulate in the body, it is released in the sweat, urine, reproductive fluids, and breath. This leads to the characteristic odor of trimethylaminuria. Affected individuals do not have other health problems related to the condition, but the unpleasant odor can result in social and psychological problems. Trimethylaminuria seems to be more common in women. Researchers think this may be due to higher hormone levels aggravating symptoms of the disorder. Trimethylaminuria is often caused by changes (mutations) in the FMO3 gene and is inherited in an autosomal recessive manner. Although there is no cure, trimethylaminuria can often be managed by reducing the amount of trimethylamine in the body. For example, affected people can modify their diet to avoid foods with high levels of trimethylamine.

MalaCards based summary : Trimethylaminuria, also known as tmau, is related to primary trimethylaminuria and noonan syndrome 1. An important gene associated with Trimethylaminuria is FMO3 (Flavin Containing Dimethylaniline Monoxygenase 3), and among its related pathways/superpathways are Drug metabolism - cytochrome P450 and Cytochrome P450 - arranged by substrate type. Affiliated tissues include liver and testes, and related phenotypes are depressivity and hypertension

Disease Ontology : 12 An inherited metabolic disorder characterized by the inabilty to break down trimethylamine and has material basis in homozygous or compound heterozygous mutation in the gene encoding flavin-containing monooxygenase-3 on chromosome 1q24.

Genetics Home Reference : 25 Trimethylaminuria is a disorder in which the body is unable to break down trimethylamine, a chemical compound that has a pungent odor. Trimethylamine has been described as smelling like rotting fish, rotting eggs, garbage, or urine. As this compound builds up in the body, it causes affected people to give off a strong odor in their sweat, urine, and breath. The intensity of the odor may vary over time. The odor can interfere with many aspects of daily life, affecting a person's relationships, social life, and career. Some people with trimethylaminuria experience depression and social isolation as a result of this condition.

OMIM : 57 Trimethylaminuria results from the abnormal presence of large amounts of volatile and malodorous trimethylamine within the body. This chemical, a tertiary aliphatic amine, is excreted in the urine, sweat (ichthyohidrosis), and breath, which take on the offensive odor of decaying fish (Mitchell, 1996). (602079)

KEGG : 37
Trimethylaminuria (TMAU), also known as fish odour syndrome, is a metabolic disorder. TMAU usually presents with a body odour resembling that of rotten or decaying fish, the result of excess excretion of trimethylamine (TMA) in the breath, sweat, urine, and reproductive fluids. Primary TMAU is most often caused by a functional defect of flavin mono-oxygenase 3 (FMO3), and the genetic disorder is inherited in an autosomal recessive manner as a consequence of mutations in the FMO3 gene. Secondary TMAU has been described in patients with severe liver disease (which is the major site of activity of the FMO3 enzyme) and chronic renal disease (as a consequence of bacterial overgrowth in the gut), and in patients treated with large doses of betaine or possibly L-carnitine. In addition, transient TMAU has been reported in a preterm infant who was fed with choline-rich food supplements and has been reported in some women just at the onset of menstruation.

UniProtKB/Swiss-Prot : 74 Trimethylaminuria: Inborn error of metabolism associated with an offensive body odor and caused by deficiency of FMO-mediated N-oxidation of amino- trimethylamine (TMA) derived from foodstuffs. Affected individuals excrete relatively large amounts of TMA in their urine, sweat, and breath, and exhibit a fishy body odor characteristic of the malodorous free amine.

Wikipedia : 75 Trimethylaminuria (TMAU), also known as fish odor syndrome or fish malodor syndrome, is a rare metabolic... more...

Related Diseases for Trimethylaminuria

Graphical network of the top 20 diseases related to Trimethylaminuria:



Diseases related to Trimethylaminuria

Symptoms & Phenotypes for Trimethylaminuria

Human phenotypes related to Trimethylaminuria:

32 (show all 8)
# Description HPO Frequency HPO Source Accession
1 depressivity 32 HP:0000716
2 hypertension 32 HP:0000822
3 splenomegaly 32 HP:0001744
4 anemia 32 HP:0001903
5 neutropenia 32 HP:0001875
6 recurrent pneumonia 32 HP:0006532
7 tachycardia 32 HP:0001649
8 trimethylaminuria 32 HP:0003614

Symptoms via clinical synopsis from OMIM:

57
Laboratory Abnormalities:
trimethylaminuria
deficiency of fmo-mediated n-oxidation of amino-trimethylamine (tma) derived from foodstuffs
large amounts of tma in urine, sweat, and breath

Hematology:
anemia (in some patients)
neutropenia in some patients)

Cardiovascular Vascular:
hypertension, severe, after eating cheese (in some patients)

Neurologic Behavioral Psychiatric Manifestations:
depression
suicidal
psychosocial problems in school

Cardiovascular Heart:
tachycardia after eating cheese (in some patients)

Respiratory Lung:
pulmonary infections (in some patients)

Clinical features from OMIM:

602079

Drugs & Therapeutics for Trimethylaminuria

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Evaluation of Gastrointestinal and Nutritional Diagnostic Tests as Potential Screening Tools for Metabolic Body Odor and Halitosis Completed NCT02692495
2 Dynamics of the Gut Microbiota in Idiopathic Malodor Production Active, not recruiting NCT03582826

Search NIH Clinical Center for Trimethylaminuria

Cochrane evidence based reviews: trimethylaminuria

Genetic Tests for Trimethylaminuria

Genetic tests related to Trimethylaminuria:

# Genetic test Affiliating Genes
1 Trimethylaminuria 29 FMO3

Anatomical Context for Trimethylaminuria

MalaCards organs/tissues related to Trimethylaminuria:

41
Liver, Testes

Publications for Trimethylaminuria

Articles related to Trimethylaminuria:

(show top 50) (show all 168)
# Title Authors PMID Year
1
Missense mutation in flavin-containing mono-oxygenase 3 gene, FMO3, underlies fish-odour syndrome. 38 8 71
9398858 1997
2
Trimethylaminuria: diet does not always control the fishy odor. 38 8 71
987532 1976
3
A novel deletion in the flavin-containing monooxygenase gene (FMO3) in a Greek patient with trimethylaminuria. 9 38 71
11266081 2001
4
Mild trimethylaminuria caused by common variants in FMO3 gene. 9 38 8
10485731 1999
5
Trimethylaminuria is caused by mutations of the FMO3 gene in a North American cohort. 9 38 71
10479479 1999
6
Two novel mutations of the FMO3 gene in a proband with trimethylaminuria. 9 38 71
10338091 1999
7
Clinical utility gene card for: trimethylaminuria. 38 71
22126753 2012
8
Primary Trimethylaminuria 38 71
20301282 2007
9
Deleterious mutations in the flavin-containing monooxygenase 3 (FMO3) gene causing trimethylaminuria. 38 71
12893987 2003
10
Fish odor syndrome. 38 8
10646019 1999
11
Transient trimethylaminuria in childhood. 38 8
9846928 1998
12
Trimethylaminuria: the detection of carriers using a trimethylamine load test. 38 8
2501587 1989
13
Trimethylaminuria ('fish-odour syndrome'): a study of an affected family. 38 8
3345632 1988
14
Trimethylaminuria (fish-odour syndrome): an inborn error of oxidative metabolism. 38 8
2881174 1987
15
Psychosocial problems as the major complication of an adolescent with trimethylaminuria. 38 8
448539 1979
16
Trimethylaminuria: fishy odors in children. 38 8
987531 1976
17
Trimethylaminuria and inherited Noonan's syndrome. 38 8
4119196 1973
18
Compound heterozygosity for missense mutations in the flavin-containing monooxygenase 3 (FM03) gene in patients with fish-odour syndrome. 71
11191884 2000
19
The fish-odor syndrome. 8
8753757 1996
20
Fish odour syndrome: verification of carrier detection test. 8
8750603 1995
21
The fish odour syndrome: biochemical, familial, and clinical aspects. 8
8401051 1993
22
Bleeding diathesis in Noonan syndrome: a common association. 8
3232698 1988
23
Trimethylamine N-oxide synthesis: a human variant. 8
5048998 1972
24
[Individual differences of drug-metabolizing enzymes as determinants for the metabolic fate of chemicals--a study of trimethylamine and flavin-containing monooxygenase 3-]. 9 38
19881207 2009
25
A novel mutation in the flavin-containing monooxygenase 3 gene (FMO3) of a Norwegian family causes trimethylaminuria. 9 38
19577495 2009
26
Novel variants of the human flavin-containing monooxygenase 3 (FMO3) gene associated with trimethylaminuria. 9 38
19321370 2009
27
Functional characterization of genetic variants of human FMO3 associated with trimethylaminuria. 9 38
17531949 2007
28
Genetic polymorphism of the flavin-containing monooxygenase 3 (FMO3) associated with trimethylaminuria (fish odor syndrome): observations from Japanese patients. 9 38
17584019 2007
29
Missense and nonsense mutations of the flavin-containing monooxygenase 3 gene in a Japanese cohort. 9 38
17329912 2007
30
Transient trimethylaminuria related to menstruation. 9 38
17257434 2007
31
Trimethylaminuria (fish-odor syndrome): a case report. 9 38
17224546 2007
32
A spectrum of molecular variation in a cohort of Italian families with trimethylaminuria: identification of three novel mutations of the FM03 gene. 9 38
16600650 2006
33
Mutation, polymorphism and perspectives for the future of human flavin-containing monooxygenase 3. 9 38
16481213 2006
34
Three novel single nucleotide polymorphisms of the FMO3 gene in a Japanese population. 9 38
16858129 2006
35
Diagnosis and management of trimethylaminuria (FMO3 deficiency) in children. 9 38
16601883 2006
36
Flavin-containing monooxygenase genetic polymorphism: impact on chemical metabolism and drug development. 9 38
16296944 2005
37
Polymorphisms of the Flavin containing monooxygenase 3 (FMO3) gene do not predispose to essential hypertension in Caucasians. 9 38
16324215 2005
38
Mammalian flavin-containing monooxygenases: structure/function, genetic polymorphisms and role in drug metabolism. 9 38
15922018 2005
39
Trimethylaminuria and a human FMO3 mutation database. 9 38
12938085 2003
40
Biochemical and clinical aspects of the human flavin-containing monooxygenase form 3 (FMO3) related to trimethylaminuria. 9 38
12678693 2003
41
Two novel single nucleotide polymorphisms (SNPs) of the FMO3 gene in Japanese. 9 38
15618753 2003
42
Interindividual differences of human flavin-containing monooxygenase 3: genetic polymorphisms and functional variation. 9 38
12228178 2002
43
Genetic polymorphisms of flavin-containing monooxygenase (FMO). 9 38
12214664 2002
44
A novel mutation in the flavin-containing monooxygenase 3 gene, FM03, that causes fish-odour syndrome: activity of the mutant enzyme assessed by proton NMR spectroscopy. 9 38
10898113 2000
45
Population-specific polymorphisms of the human FMO3 gene: significance for detoxication. 9 38
10640514 2000
46
Sequence variations in the flavin-containing mono-oxygenase 3 gene (FMO3) in fish odour syndrome. 9 38
10215790 1999
47
Mutations of the flavin-containing monooxygenase gene (FMO3) cause trimethylaminuria, a defect in detoxication. 9 38
9536088 1998
48
Structural organization of the human flavin-containing monooxygenase 3 gene (FMO3), the favored candidate for fish-odor syndrome, determined directly from genomic DNA. 9 38
9417913 1997
49
Human flavin-containing monooxygenase form 3: cDNA expression of the enzymes containing amino acid substitutions observed in individuals with trimethylaminuria. 9 38
9282831 1997
50
Novel variants and haplotypes of human flavin-containing monooxygenase 3 gene associated with Japanese subjects suffering from trimethylaminuria. 38
30351217 2019

Variations for Trimethylaminuria

ClinVar genetic disease variations for Trimethylaminuria:

6 (show all 45)
# Gene Variation Type Significance SNP ID GRCh37 Pos GRCh38 Pos
1 FMO3 NM_001002294.3(FMO3): c.1079T> C (p.Leu360Pro) single nucleotide variant other rs28363581 1:171083398-171083398 1:171114258-171114258
2 FMO3 NM_001002294.3(FMO3): c.913G> T (p.Glu305Ter) single nucleotide variant Pathogenic rs61753344 1:171083232-171083232 1:171114092-171114092
3 FMO3 FMO3, 2-EX DEL deletion Pathogenic
4 FMO3 NM_001002294.3(FMO3): c.94G> A (p.Glu32Lys) single nucleotide variant Pathogenic rs72549320 1:171061893-171061893 1:171092752-171092752
5 FMO3 FMO3, 1-BP DEL, 191A deletion Pathogenic
6 FMO3 NM_001002294.3(FMO3): c.198G> T (p.Met66Ile) single nucleotide variant Pathogenic rs72549323 1:171072991-171072991 1:171103850-171103850
7 FMO3 NM_001002294.3(FMO3): c.154G> A (p.Ala52Thr) single nucleotide variant Pathogenic rs72549321 1:171072947-171072947 1:171103806-171103806
8 FMO3 NM_001002294.3(FMO3): c.458C> T (p.Pro153Leu) single nucleotide variant Pathogenic rs72549326 1:171076952-171076952 1:171107811-171107811
9 FMO3 NM_001002294.3(FMO3): c.1160G> T (p.Arg387Leu) single nucleotide variant Pathogenic rs72549331 1:171083479-171083479 1:171114339-171114339
10 FMO3 NM_001002294.3(FMO3): c.940G> T (p.Glu314Ter) single nucleotide variant Pathogenic rs72549330 1:171083259-171083259 1:171114119-171114119
11 FMO3 NM_001002294.3(FMO3): c.182A> G (p.Asn61Ser) single nucleotide variant Pathogenic rs72549322 1:171072975-171072975 1:171103834-171103834
12 FMO3 NM_001002294.3(FMO3): c.589_590TG[1] (p.Cys197_Asp198delinsTer) short repeat Pathogenic rs3832024 1:171077326-171077327 1:171108185-171108186
13 FMO3 NM_001002294.3(FMO3): c.442G> A (p.Gly148Arg) single nucleotide variant Likely pathogenic 1:171076936-171076936 1:171107795-171107795
14 FMO3 NM_001002294.3(FMO3): c.370C> T (p.Gln124Ter) single nucleotide variant Likely pathogenic 1:171076864-171076864 1:171107723-171107723
15 FMO3 NM_001002294.3(FMO3): c.929C> T (p.Ser310Leu) single nucleotide variant Likely pathogenic 1:171083248-171083248 1:171114108-171114108
16 FMO3 NM_001002294.3(FMO3): c.1474C> T (p.Arg492Trp) single nucleotide variant Likely pathogenic rs72549334 1:171086457-171086457 1:171117317-171117317
17 FMO3 NM_001002294.3(FMO3): c.1221T> C (p.Asn407=) single nucleotide variant Conflicting interpretations of pathogenicity rs79553697 1:171085385-171085385 1:171116245-171116245
18 FMO3 NM_001002294.3(FMO3): c.260T> A (p.Ile87Asn) single nucleotide variant Uncertain significance rs886045559 1:171073053-171073053 1:171103912-171103912
19 FMO3 NM_001002294.3(FMO3): c.994A> G (p.Ser332Gly) single nucleotide variant Uncertain significance rs886045561 1:171083313-171083313 1:171114173-171114173
20 FMO3 NM_001002294.3(FMO3): c.1204A> G (p.Met402Val) single nucleotide variant Uncertain significance rs141117096 1:171085368-171085368 1:171116228-171116228
21 FMO3 NM_001002294.3(FMO3): c.225C> T (p.Pro75=) single nucleotide variant Uncertain significance rs141235954 1:171073018-171073018 1:171103877-171103877
22 FMO3 NM_001002294.3(FMO3): c.889G> T (p.Val297Leu) single nucleotide variant Uncertain significance rs762874376 1:171083208-171083208 1:171114068-171114068
23 FMO3 NM_001002294.3(FMO3): c.1505T> G (p.Val502Gly) single nucleotide variant Uncertain significance rs60306057 1:171086488-171086488 1:171117348-171117348
24 FMO3 NM_001002294.3(FMO3): c.*364C> T single nucleotide variant Uncertain significance rs771349442 1:171086946-171086946 1:171117806-171117806
25 FMO3 NM_001002294.3(FMO3): c.*52G> A single nucleotide variant Uncertain significance rs777464034 1:171086634-171086634 1:171117494-171117494
26 FMO3 NM_001002294.3(FMO3): c.*374A> G single nucleotide variant Uncertain significance rs187172674 1:171086956-171086956 1:171117816-171117816
27 FMO3 NM_001002294.3(FMO3): c.-25G> A single nucleotide variant Uncertain significance rs11578281 1:171060104-171060104 1:171090963-171090963
28 FMO3 NM_001002294.3(FMO3): c.430A> G (p.Met144Val) single nucleotide variant Uncertain significance rs886045560 1:171076924-171076924 1:171107783-171107783
29 FMO3 NM_001002294.3(FMO3): c.245T> C (p.Met82Thr) single nucleotide variant Uncertain significance rs72549324 1:171073038-171073038 1:171103897-171103897
30 FMO3 NM_001002294.3(FMO3): c.979T> G (p.Phe327Val) single nucleotide variant Uncertain significance rs767834931 1:171083298-171083298 1:171114158-171114158
31 FMO3 NM_001002294.3(FMO3): c.1288T> C (p.Tyr430His) single nucleotide variant Uncertain significance rs756249764 1:171086271-171086271 1:171117131-171117131
32 FMO3 NM_001002294.3(FMO3): c.1539T> C (p.His513=) single nucleotide variant Uncertain significance rs771817026 1:171086522-171086522 1:171117382-171117382
33 FMO3 NM_001002294.3(FMO3): c.628-6T> C single nucleotide variant Uncertain significance rs369396459 1:171079933-171079933 1:171110792-171110792
34 FMO3 NM_001002294.3(FMO3): c.706G> A (p.Val236Ile) single nucleotide variant Uncertain significance rs201271626 1:171080017-171080017 1:171110876-171110876
35 FMO3 NM_001002294.3(FMO3): c.1302G> A (p.Met434Ile) single nucleotide variant Uncertain significance rs72549332 1:171086285-171086285 1:171117145-171117145
36 FMO3 NM_001002294.3(FMO3): c.1123dup (p.Leu375fs) duplication Uncertain significance 1:171083439-171083439 1:171114302-171114302
37 FMO3 NM_001002294.3(FMO3): c.172G> A (p.Val58Ile) single nucleotide variant Uncertain significance rs144935285 1:171072965-171072965 1:171103824-171103824
38 FMO3 NM_001002294.3(FMO3): c.830T> C (p.Val277Ala) single nucleotide variant Benign/Likely benign rs2066530 1:171083149-171083149 1:171114009-171114009
39 FMO3 NM_001002294.3(FMO3): c.769G> A (p.Val257Met) single nucleotide variant Benign/Likely benign rs1736557 1:171080080-171080080 1:171110939-171110939
40 FMO3 NM_006894.5(FMO3): c.472G> A (p.Glu158Lys) single nucleotide variant Benign rs2266782 1:171076966-171076966 1:171107825-171107825
41 FMO3 NM_006894.5(FMO3): c.923A> G (p.Glu308Gly) single nucleotide variant Benign rs2266780 1:171083242-171083242 1:171114102-171114102
42 FMO3 NM_001002294.3(FMO3): c.855C> T (p.Asn285=) single nucleotide variant Benign rs909530 1:171083174-171083174 1:171114034-171114034
43 FMO3 NM_001002294.3(FMO3): c.441C> T (p.Ser147=) single nucleotide variant Benign rs1800822 1:171076935-171076935 1:171107794-171107794
44 FMO3 NM_001002294.3(FMO3): c.627+10C> G single nucleotide variant Benign rs2066534 1:171077372-171077372 1:171108231-171108231
45 FMO3 NM_001002294.3(FMO3): c.560T> C (p.Val187Ala) single nucleotide variant no interpretation for the single variant rs769983049 1:171077295-171077295 1:171108154-171108154

UniProtKB/Swiss-Prot genetic disease variations for Trimethylaminuria:

74
# Symbol AA change Variation ID SNP ID
1 FMO3 p.Met66Ile VAR_002423 rs72549323
2 FMO3 p.Pro153Leu VAR_002424 rs72549326
3 FMO3 p.Arg492Trp VAR_008145 rs72549334
4 FMO3 p.Ala52Thr VAR_008146 rs72549321
5 FMO3 p.Arg387Leu VAR_008147 rs72549331
6 FMO3 p.Glu32Lys VAR_037306 rs72549320
7 FMO3 p.Asn61Ser VAR_037307 rs72549322
8 FMO3 p.Met434Ile VAR_037308 rs72549332

Expression for Trimethylaminuria

Search GEO for disease gene expression data for Trimethylaminuria.

Pathways for Trimethylaminuria

Pathways related to Trimethylaminuria according to KEGG:

37
# Name Kegg Source Accession
1 Drug metabolism - cytochrome P450 hsa00982

Pathways related to Trimethylaminuria according to GeneCards Suite gene sharing:

(show all 16)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.86 FMO5 FMO4 FMO3 FMO2 FMO1 CYP2E1
2
Show member pathways
12.49 FMO5 FMO4 FMO3 FMO2 FMO1 CYP2E1
3 11.9 FMO3 C4B C4A
4 11.54 C4B C4A
5
Show member pathways
11.48 CYP2E1 CYP2B6 CYP2A6
6
Show member pathways
11.35 CYP2E1 CYP2B6 CYP2A6
7 11.09 CYP2E1 CYP2B6
8 11.04 CYP2B6 CYP2A6
9
Show member pathways
11 FMO5 FMO4 FMO3 FMO2 FMO1
10
Show member pathways
10.94 CYP2E1 CYP2A6
11 10.87 CYP2E1 CYP2A6
12 10.78 CYP2E1 CYP2A6
13
Show member pathways
10.76 CYP2B6 CYP2A6
14
Show member pathways
10.73 FMO5 FMO3 CYP2A6
15
Show member pathways
10.61 FMO3 FMO1 CYP2E1 CYP2B6 CYP2A6
16
Show member pathways
10.47 CYP2E1 CYP2A6

GO Terms for Trimethylaminuria

Cellular components related to Trimethylaminuria according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 endoplasmic reticulum GO:0005783 9.86 FMO5 FMO4 FMO3 FMO2 FMO1 CYP2E1
2 endoplasmic reticulum membrane GO:0005789 9.76 FMO5 FMO4 FMO3 FMO2 FMO1 CYP2E1
3 intracellular membrane-bounded organelle GO:0043231 9.56 FMO5 FMO4 FMO3 FMO2 FMO1 CYP2E1
4 organelle membrane GO:0031090 9.23 FMO5 FMO4 FMO3 FMO2 FMO1 CYP2E1

Biological processes related to Trimethylaminuria according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 oxidation-reduction process GO:0055114 9.86 FMO5 FMO4 FMO3 FMO2 FMO1 CYP2E1
2 steroid metabolic process GO:0008202 9.65 CYP2E1 CYP2B6 CYP2A6
3 xenobiotic metabolic process GO:0006805 9.63 FMO3 FMO2 FMO1 CYP2E1 CYP2B6 CYP2A6
4 exogenous drug catabolic process GO:0042738 9.54 CYP2E1 CYP2B6 CYP2A6
5 epoxygenase P450 pathway GO:0019373 9.5 CYP2E1 CYP2B6 CYP2A6
6 toxin metabolic process GO:0009404 9.46 FMO2 FMO1
7 positive regulation of apoptotic cell clearance GO:2000427 9.43 C4B C4A
8 NADPH oxidation GO:0070995 9.4 FMO2 FMO1
9 drug metabolic process GO:0017144 9.35 FMO2 FMO1 CYP2E1 CYP2B6 CYP2A6
10 organic acid metabolic process GO:0006082 9.02 FMO2 FMO1 CYP2E1 CYP2B6 CYP2A6

Molecular functions related to Trimethylaminuria according to GeneCards Suite gene sharing:

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 oxidoreductase activity GO:0016491 9.86 FMO5 FMO4 FMO3 FMO2 FMO1 CYP2E1
2 flavin adenine dinucleotide binding GO:0050660 9.72 FMO5 FMO4 FMO3 FMO2 FMO1
3 iron ion binding GO:0005506 9.67 CYP2E1 CYP2B6 CYP2A6
4 heme binding GO:0020037 9.65 CYP2E1 CYP2B6 CYP2A6
5 oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen GO:0016705 9.63 CYP2E1 CYP2B6 CYP2A6
6 steroid hydroxylase activity GO:0008395 9.61 CYP2E1 CYP2B6 CYP2A6
7 oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen GO:0016712 9.58 CYP2E1 CYP2B6 CYP2A6
8 NADP binding GO:0050661 9.55 FMO5 FMO4 FMO3 FMO2 FMO1
9 arachidonic acid epoxygenase activity GO:0008392 9.54 CYP2E1 CYP2B6 CYP2A6
10 endopeptidase inhibitor activity GO:0004866 9.51 C4B C4A
11 monooxygenase activity GO:0004497 9.5 FMO5 FMO4 FMO3 FMO2 FMO1 CYP2E1
12 N,N-dimethylaniline monooxygenase activity GO:0004499 9.02 FMO5 FMO4 FMO3 FMO2 FMO1

Sources for Trimethylaminuria

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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