TMAU
MCID: TRM004
MIFTS: 45

Trimethylaminuria (TMAU)

Categories: Genetic diseases, Metabolic diseases, Rare diseases

Aliases & Classifications for Trimethylaminuria

MalaCards integrated aliases for Trimethylaminuria:

Name: Trimethylaminuria 58 12 77 54 26 76 38 30 13 56 6 15 74
Tmau 58 54 26 76
Fish-Odor Syndrome 58 12 76
Fish Malodor Syndrome 54 26
Stale Fish Syndrome 54 26
Fish Odor Syndrome 54 26
Tmauria 54 26
Severe Primary Trimethylaminuria 60
Trimethylaminuria ) 41
Mesh; D008661 76

Characteristics:

OMIM:

58
Inheritance:
autosomal recessive

Miscellaneous:
offensive fishy body odor


HPO:

33
trimethylaminuria:
Inheritance autosomal recessive inheritance


Classifications:

Orphanet: 60  
Inborn errors of metabolism


Summaries for Trimethylaminuria

NIH Rare Diseases : 54 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 depression. 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 testes and temporal lobe, 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 : 26 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 : 58 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)

UniProtKB/Swiss-Prot : 76 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 : 77 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:

33 (show all 8)
# Description HPO Frequency HPO Source Accession
1 depressivity 33 HP:0000716
2 hypertension 33 HP:0000822
3 splenomegaly 33 HP:0001744
4 anemia 33 HP:0001903
5 neutropenia 33 HP:0001875
6 recurrent pneumonia 33 HP:0006532
7 tachycardia 33 HP:0001649
8 trimethylaminuria 33 HP:0003614

Symptoms via clinical synopsis from OMIM:

58
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)

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

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

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

Clinical features from OMIM:

602079

Drugs & Therapeutics for Trimethylaminuria

Search Clinical Trials , NIH Clinical Center for Trimethylaminuria

Genetic Tests for Trimethylaminuria

Genetic tests related to Trimethylaminuria:

# Genetic test Affiliating Genes
1 Trimethylaminuria 30 FMO3

Anatomical Context for Trimethylaminuria

MalaCards organs/tissues related to Trimethylaminuria:

42
Testes, Temporal Lobe

Publications for Trimethylaminuria

Articles related to Trimethylaminuria:

(show top 50) (show all 95)
# Title Authors Year
1
Development and feasibility of the use of an assessment tool measuring treatment efficacy in patients with trimethylaminuria: A mixed methods study. ( 30734325 )
2019
2
Novel variants and haplotypes of human flavin-containing monooxygenase 3 gene associated with Japanese subjects suffering from trimethylaminuria. ( 30351217 )
2018
3
Primary trimethylaminuria (fish odor syndrome) and hypothyroidism in an adolescent. ( 29745129 )
2017
4
Primary and transitory trimethylaminuria: A report of 2 cases. ( 28743400 )
2017
5
A compound heterozygous mutation in the FMO3 gene: the first pediatric case causes fish odor syndrome in Korea. ( 28392825 )
2017
6
First case of Currarino syndrome and trimethylaminuria: two rare diseases for a complex clinical presentation. ( 27335202 )
2016
7
Trimethylaminuria. ( 27118741 )
2016
8
Clinical utility gene card for: Trimethylaminuria - update 2014. ( 25335494 )
2015
9
Analysis of six novel flavin-containing monooxygenase 3 (FMO3) gene variants found in a Japanese population suffering from trimethylaminuria. ( 28649550 )
2015
10
Transient massive trimethylaminuria associated with food protein-induced enterocolitis syndrome. ( 23821320 )
2014
11
Relationships between flavin-containing mono-oxygenase 3 (FMO3) genotype and trimethylaminuria phenotype in a Japanese population. ( 24028545 )
2014
12
Archaebiotics: proposed therapeutic use of archaea to prevent trimethylaminuria and cardiovascular disease. ( 24247281 )
2014
13
Fish odor syndrome: a case report of trimethylaminuria. ( 24456963 )
2014
14
Fish odor syndrome (trimethylaminuria) supporting the possible FMO3 down expression in childhood: a case report. ( 25288227 )
2014
15
A rare case of fish odor syndrome presenting as depression. ( 24891709 )
2014
16
Delusional disorder, somatic type: olfactory reference syndrome in a patient with delusional trimethylaminuria. ( 23719328 )
2013
17
FMO3 allelic variants in Sicilian and Sardinian populations: trimethylaminuria and absence of fish-like body odor. ( 23266626 )
2013
18
Trimethylaminuria (fish odor syndrome): genotype characterization among Portuguese patients. ( 23791655 )
2013
19
A review of trimethylaminuria: (fish odor syndrome). ( 24307925 )
2013
20
Riboflavin-responsive trimethylaminuria in a patient with homocystinuria on betaine therapy. ( 23430919 )
2012
21
Trimethylaminuria: an under-recognised and socially debilitating metabolic disorder. ( 21276117 )
2012
22
Clinical utility gene card for: trimethylaminuria. ( 22126753 )
2012
23
Variants in the flavin-containing monooxygenase 3 (FMO3) gene responsible for trimethylaminuria in a Japanese population. ( 22819296 )
2012
24
Trimethylaminuria (fish malodour syndrome) in chronic renal failure. ( 23930066 )
2012
25
Epilepsy and trimethylaminuria: A new case report and literature review. ( 20970269 )
2011
26
Trimethylaminuria: causes and diagnosis of a socially distressing condition. ( 21451776 )
2011
27
Individuals reporting idiopathic malodor production: demographics and incidence of trimethylaminuria. ( 21851918 )
2011
28
[Fish odour--could be a sign of trimethylaminuria]. ( 21092725 )
2010
29
Novel variants of the human flavin-containing monooxygenase 3 (FMO3) gene associated with trimethylaminuria. ( 19321370 )
2009
30
A novel mutation in the flavin-containing monooxygenase 3 gene (FMO3) of a Norwegian family causes trimethylaminuria. ( 19577495 )
2009
31
Bonitos with low content of malodorous trimethylamine as palliative care for self-reported Japanese trimethylaminuria subjects. ( 20045990 )
2009
32
Perioperative recommendations in a child with Fish Odor syndrome. ( 19691710 )
2009
33
[Primary trimethylaminuria: the fish odor syndrome]. ( 19695515 )
2009
34
Trimethylaminuria (fish malodour syndrome): a "benign" genetic condition with major psychosocial sequelae. ( 18928446 )
2008
35
Stop codon mutations in the flavin-containing monooxygenase 3 (FMO3) gene responsible for trimethylaminuria in a Japanese population. ( 16996766 )
2007
36
Trimethylaminuria (fish-odor syndrome): a case report. ( 17224546 )
2007
37
Transient trimethylaminuria related to menstruation. ( 17257434 )
2007
38
Rapid screening assay of trimethylaminuria in urine with matrix-assisted laser desorption/ionization time-of-flight mass spectrometry. ( 17510942 )
2007
39
Functional characterization of genetic variants of human FMO3 associated with trimethylaminuria. ( 17531949 )
2007
40
Genetic polymorphism of the flavin-containing monooxygenase 3 (FMO3) associated with trimethylaminuria (fish odor syndrome): observations from Japanese patients. ( 17584019 )
2007
41
A spectrum of molecular variation in a cohort of Italian families with trimethylaminuria: identification of three novel mutations of the FM03 gene. ( 16600650 )
2006
42
Diagnosis and management of trimethylaminuria (FMO3 deficiency) in children. ( 16601883 )
2006
43
Smelling like dead fish: a case of trimethylaminuria in an adolescent. ( 17041178 )
2006
44
Diagnosis of suspected trimethylaminuria by NMR spectroscopy. ( 15563884 )
2005
45
Trimethylaminuria (fish-odour syndrome) and oral malodour. ( 15752091 )
2005
46
Mild trimethylaminuria observed in a Japanese cohort with liver damage. ( 15989923 )
2005
47
Effects of the dietary supplements, activated charcoal and copper chlorophyllin, on urinary excretion of trimethylamine in Japanese trimethylaminuria patients. ( 15043988 )
2004
48
Choline- and betaine-defined diets for use in clinical research and for the management of trimethylaminuria. ( 15565078 )
2004
49
Fish odour syndrome with features of both primary and secondary trimethylaminuria. ( 12653714 )
2003
50
Biochemical and clinical aspects of the human flavin-containing monooxygenase form 3 (FMO3) related to trimethylaminuria. ( 12678693 )
2003

Variations for Trimethylaminuria

UniProtKB/Swiss-Prot genetic disease variations for Trimethylaminuria:

76
# 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

ClinVar genetic disease variations for Trimethylaminuria:

6 (show top 50) (show all 84)
# Gene Variation Type Significance SNP ID Assembly Location
1 FMO3 NM_006894.5(FMO3): c.913G> T (p.Glu305Ter) single nucleotide variant Pathogenic rs61753344 GRCh37 Chromosome 1, 171083232: 171083232
2 FMO3 NM_006894.5(FMO3): c.913G> T (p.Glu305Ter) single nucleotide variant Pathogenic rs61753344 GRCh38 Chromosome 1, 171114092: 171114092
3 FMO3 NM_006894.5(FMO3): c.769G> A (p.Val257Met) single nucleotide variant Conflicting interpretations of pathogenicity rs1736557 GRCh37 Chromosome 1, 171080080: 171080080
4 FMO3 NM_006894.5(FMO3): c.769G> A (p.Val257Met) single nucleotide variant Conflicting interpretations of pathogenicity rs1736557 GRCh38 Chromosome 1, 171110939: 171110939
5 FMO3 NM_001002294.2(FMO3): c.198G> T (p.Met66Ile) single nucleotide variant Pathogenic rs72549323 GRCh37 Chromosome 1, 171072991: 171072991
6 FMO3 NM_001002294.2(FMO3): c.198G> T (p.Met66Ile) single nucleotide variant Pathogenic rs72549323 GRCh38 Chromosome 1, 171103850: 171103850
7 FMO3 NM_001002294.2(FMO3): c.154G> A (p.Ala52Thr) single nucleotide variant Pathogenic rs72549321 GRCh37 Chromosome 1, 171072947: 171072947
8 FMO3 NM_001002294.2(FMO3): c.154G> A (p.Ala52Thr) single nucleotide variant Pathogenic rs72549321 GRCh38 Chromosome 1, 171103806: 171103806
9 FMO3 NM_006894.5(FMO3): c.458C> T (p.Pro153Leu) single nucleotide variant Pathogenic rs72549326 GRCh37 Chromosome 1, 171076952: 171076952
10 FMO3 NM_006894.5(FMO3): c.458C> T (p.Pro153Leu) single nucleotide variant Pathogenic rs72549326 GRCh38 Chromosome 1, 171107811: 171107811
11 FMO3 NM_006894.5(FMO3): c.1474C> T (p.Arg492Trp) single nucleotide variant Likely pathogenic rs72549334 GRCh37 Chromosome 1, 171086457: 171086457
12 FMO3 NM_006894.5(FMO3): c.1474C> T (p.Arg492Trp) single nucleotide variant Likely pathogenic rs72549334 GRCh38 Chromosome 1, 171117317: 171117317
13 FMO3 NM_001002294.2(FMO3): c.1160G> T (p.Arg387Leu) single nucleotide variant Pathogenic rs72549331 GRCh37 Chromosome 1, 171083479: 171083479
14 FMO3 NM_001002294.2(FMO3): c.1160G> T (p.Arg387Leu) single nucleotide variant Pathogenic rs72549331 GRCh38 Chromosome 1, 171114339: 171114339
15 FMO3 NM_001002294.2(FMO3): c.940G> T (p.Glu314Ter) single nucleotide variant Pathogenic rs72549330 GRCh37 Chromosome 1, 171083259: 171083259
16 FMO3 NM_001002294.2(FMO3): c.940G> T (p.Glu314Ter) single nucleotide variant Pathogenic rs72549330 GRCh38 Chromosome 1, 171114119: 171114119
17 FMO3 NM_006894.5(FMO3): c.182A> G (p.Asn61Ser) single nucleotide variant Pathogenic rs72549322 GRCh37 Chromosome 1, 171072975: 171072975
18 FMO3 NM_006894.5(FMO3): c.182A> G (p.Asn61Ser) single nucleotide variant Pathogenic rs72549322 GRCh38 Chromosome 1, 171103834: 171103834
19 FMO3 NM_001002294.2(FMO3): c.1302G> A (p.Met434Ile) single nucleotide variant Pathogenic rs72549332 GRCh37 Chromosome 1, 171086285: 171086285
20 FMO3 NM_001002294.2(FMO3): c.1302G> A (p.Met434Ile) single nucleotide variant Pathogenic rs72549332 GRCh38 Chromosome 1, 171117145: 171117145
21 FMO3 FMO3, 2-EX DEL deletion Pathogenic
22 FMO3 NM_001002294.2(FMO3): c.94G> A (p.Glu32Lys) single nucleotide variant Pathogenic rs72549320 GRCh37 Chromosome 1, 171061893: 171061893
23 FMO3 NM_001002294.2(FMO3): c.94G> A (p.Glu32Lys) single nucleotide variant Pathogenic rs72549320 GRCh38 Chromosome 1, 171092752: 171092752
24 FMO3 FMO3, 1-BP DEL, 191A deletion Pathogenic
25 FMO3 NM_006894.5(FMO3): c.923A> G (p.Glu308Gly) single nucleotide variant Benign rs2266780 GRCh37 Chromosome 1, 171083242: 171083242
26 FMO3 NM_006894.5(FMO3): c.923A> G (p.Glu308Gly) single nucleotide variant Benign rs2266780 GRCh38 Chromosome 1, 171114102: 171114102
27 FMO3 NM_006894.5(FMO3): c.1079T> C (p.Leu360Pro) single nucleotide variant other rs28363581 GRCh37 Chromosome 1, 171083398: 171083398
28 FMO3 NM_006894.5(FMO3): c.1079T> C (p.Leu360Pro) single nucleotide variant other rs28363581 GRCh38 Chromosome 1, 171114258: 171114258
29 FMO3 NM_006894.5(FMO3): c.472G> A (p.Glu158Lys) single nucleotide variant Benign rs2266782 GRCh37 Chromosome 1, 171076966: 171076966
30 FMO3 NM_006894.5(FMO3): c.472G> A (p.Glu158Lys) single nucleotide variant Benign rs2266782 GRCh38 Chromosome 1, 171107825: 171107825
31 FMO3 NM_006894.5(FMO3): c.560T> C (p.Val187Ala) single nucleotide variant no interpretation for the single variant rs769983049 GRCh37 Chromosome 1, 171077295: 171077295
32 FMO3 NM_006894.5(FMO3): c.560T> C (p.Val187Ala) single nucleotide variant no interpretation for the single variant rs769983049 GRCh38 Chromosome 1, 171108154: 171108154
33 FMO3 NM_006894.5(FMO3): c.172G> A (p.Val58Ile) single nucleotide variant Uncertain significance rs144935285 GRCh37 Chromosome 1, 171072965: 171072965
34 FMO3 NM_006894.5(FMO3): c.172G> A (p.Val58Ile) single nucleotide variant Uncertain significance rs144935285 GRCh38 Chromosome 1, 171103824: 171103824
35 FMO3 NM_006894.5(FMO3): c.591_592delTG (p.Cys197Terfs) deletion Pathogenic rs3832024 GRCh37 Chromosome 1, 171077326: 171077327
36 FMO3 NM_006894.5(FMO3): c.591_592delTG (p.Cys197Terfs) deletion Pathogenic rs3832024 GRCh38 Chromosome 1, 171108185: 171108186
37 FMO3 NM_006894.5(FMO3): c.441C> T (p.Ser147=) single nucleotide variant Benign rs1800822 GRCh37 Chromosome 1, 171076935: 171076935
38 FMO3 NM_006894.5(FMO3): c.441C> T (p.Ser147=) single nucleotide variant Benign rs1800822 GRCh38 Chromosome 1, 171107794: 171107794
39 FMO3 NM_006894.5(FMO3): c.627+10C> G single nucleotide variant Benign rs2066534 GRCh37 Chromosome 1, 171077372: 171077372
40 FMO3 NM_006894.5(FMO3): c.627+10C> G single nucleotide variant Benign rs2066534 GRCh38 Chromosome 1, 171108231: 171108231
41 FMO3 NM_006894.5(FMO3): c.830T> C (p.Val277Ala) single nucleotide variant Benign/Likely benign rs2066530 GRCh38 Chromosome 1, 171114009: 171114009
42 FMO3 NM_006894.5(FMO3): c.830T> C (p.Val277Ala) single nucleotide variant Benign/Likely benign rs2066530 GRCh37 Chromosome 1, 171083149: 171083149
43 FMO3 NM_006894.5(FMO3): c.855C> T (p.Asn285=) single nucleotide variant Benign rs909530 GRCh38 Chromosome 1, 171114034: 171114034
44 FMO3 NM_006894.5(FMO3): c.855C> T (p.Asn285=) single nucleotide variant Benign rs909530 GRCh37 Chromosome 1, 171083174: 171083174
45 FMO3 NM_006894.5(FMO3): c.1221T> C (p.Asn407=) single nucleotide variant Conflicting interpretations of pathogenicity rs79553697 GRCh37 Chromosome 1, 171085385: 171085385
46 FMO3 NM_006894.5(FMO3): c.1221T> C (p.Asn407=) single nucleotide variant Conflicting interpretations of pathogenicity rs79553697 GRCh38 Chromosome 1, 171116245: 171116245
47 FMO3 NM_006894.5(FMO3): c.260T> A (p.Ile87Asn) single nucleotide variant Uncertain significance rs886045559 GRCh38 Chromosome 1, 171103912: 171103912
48 FMO3 NM_006894.5(FMO3): c.260T> A (p.Ile87Asn) single nucleotide variant Uncertain significance rs886045559 GRCh37 Chromosome 1, 171073053: 171073053
49 FMO3 NM_006894.5(FMO3): c.994A> G (p.Ser332Gly) single nucleotide variant Uncertain significance rs886045561 GRCh38 Chromosome 1, 171114173: 171114173
50 FMO3 NM_006894.5(FMO3): c.994A> G (p.Ser332Gly) single nucleotide variant Uncertain significance rs886045561 GRCh37 Chromosome 1, 171083313: 171083313

Expression for Trimethylaminuria

Search GEO for disease gene expression data for Trimethylaminuria.

Pathways for Trimethylaminuria

Pathways related to Trimethylaminuria according to KEGG:

38
# Name Kegg Source Accession
1 Drug metabolism - cytochrome P450 hsa00982

Pathways related to Trimethylaminuria according to GeneCards Suite gene sharing:

(show all 11)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.87 CYP2A6 CYP2E1 FMO1 FMO2 FMO3 FMO4
2
Show member pathways
12.23 CYP2A6 CYP2E1 FMO1 FMO2 FMO3 FMO4
3 11.87 C4A C4B FMO3
4
Show member pathways
11.67 CYP2E1 FMO1 FMO3
5
Show member pathways
11.05 CYP2A6 CYP2E1 FMO1 FMO3
6
Show member pathways
10.97 CYP2A6 CYP2E1
7 10.84 CYP2A6 CYP2E1
8 10.73 CYP2A6 CYP2E1
9
Show member pathways
10.66 CYP2A6 FMO3 FMO5
10
Show member pathways
10.66 FMO1 FMO2 FMO3 FMO4 FMO5
11
Show member pathways
10.45 CYP2A6 CYP2E1

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 9.8 CYP2A6 CYP2E1 FMO1 FMO2 FMO3 FMO4
2 endoplasmic reticulum membrane GO:0005789 9.7 CYP2A6 CYP2E1 FMO1 FMO2 FMO3 FMO4
3 intracellular membrane-bounded organelle GO:0043231 9.5 CYP2A6 CYP2E1 FMO1 FMO2 FMO3 FMO4
4 organelle membrane GO:0031090 9.17 CYP2A6 CYP2E1 FMO1 FMO2 FMO3 FMO4

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 CYP2A6 CYP2E1 DECR1 FMO1 FMO2 FMO3
2 xenobiotic metabolic process GO:0006805 9.55 CYP2A6 CYP2E1 FMO1 FMO2 FMO3
3 exogenous drug catabolic process GO:0042738 9.46 CYP2A6 CYP2E1
4 epoxygenase P450 pathway GO:0019373 9.43 CYP2A6 CYP2E1
5 toxin metabolic process GO:0009404 9.4 FMO1 FMO2
6 positive regulation of apoptotic cell clearance GO:2000427 9.37 C4A C4B
7 NADPH oxidation GO:0070995 9.32 FMO1 FMO2
8 drug metabolic process GO:0017144 9.26 CYP2A6 CYP2E1 FMO1 FMO2
9 organic acid metabolic process GO:0006082 8.92 CYP2A6 CYP2E1 FMO1 FMO2

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

# Name GO ID Score Top Affiliating Genes
1 oxidoreductase activity GO:0016491 9.86 CYP2A6 CYP2E1 DECR1 FMO1 FMO2 FMO3
2 flavin adenine dinucleotide binding GO:0050660 9.72 FMO1 FMO2 FMO3 FMO4 FMO5
3 NADP binding GO:0050661 9.55 FMO1 FMO2 FMO3 FMO4 FMO5
4 endopeptidase inhibitor activity GO:0004866 9.46 C4A C4B
5 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 9.43 CYP2A6 CYP2E1
6 monooxygenase activity GO:0004497 9.43 CYP2E1 FMO1 FMO2 FMO3 FMO4 FMO5
7 steroid hydroxylase activity GO:0008395 9.4 CYP2A6 CYP2E1
8 arachidonic acid epoxygenase activity GO:0008392 9.37 CYP2A6 CYP2E1
9 N,N-dimethylaniline monooxygenase activity GO:0004499 9.02 FMO1 FMO2 FMO3 FMO4 FMO5

Sources for Trimethylaminuria

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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