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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-10-13 22:07:39 UTC
HMDB IDHMDB00696
Secondary Accession NumbersNone
Metabolite Identification
Common NameL-Methionine
DescriptionMethionine is a dietary indispensable amino acid required for normal growth and development of humans, other mammals, and avian species. In addition to being a substrate for protein synthesis, it is an intermediate in transmethylation reactions, serving as the major methyl group donor in vivo, including the methyl groups for DNA and RNA intermediates. Methionine is a methyl acceptor for 5-methyltetrahydrofolate-homocysteine methyl transferase (methionine synthase), the only reaction that allows for the recycling of this form of folate, and is also a methyl acceptor for the catabolism of betaine. Methionine is also required for synthesis of cysteine. Methionine is accepted as the metabolic precursor for cysteine. Only the sulfur atom from methionine is transferred to cysteine; the carbon skeleton of cysteine is donated by serine. (PMID 16702340 ). The adequacy range of dietary requirements of specific amino acids in disease states is difficult to determine. Requirements may not be similar in disease with regard to protein synthesis. Requirements for this purpose can be assessed only when such a function can be measured and related to clinical outcome. There is apparent consensus concerning normal sulfur amino acid (SAA) requirements. WHO recommendations amount to 13 mg/kg per 24 h in healthy adults. This amount is roughly doubled in artificial nutrition regimens. In disease or after trauma, requirements may be altered for methionine, cysteine, and taurine. Although in specific cases of congenital enzyme deficiency, prematurity, or diminished liver function, hypermethionemia or hyperhomocysteinemia may occur, SAA supplementation can be considered safe in amounts exceeding 2-3 times the minimal recommended daily intake. Apart from some very specific indications (e.g., acetaminophen poisoning) the usefulness of SAA supplementation is not yet established.(PMID 16702341 ). Methionine is known to exacerbate psychopathological symptoms in schizophrenic patients, there is no evidence of similar effects in healthy subjects. The role of methionine as a precursor of homocysteine is the most notable cause for concern. A "loading dose" of methionine (0.1 g/kg) has been given, and the resultant acute increase in plasma homocysteine has been used as an index of the susceptibility to cardiovascular disease. Although this procedure results in vascular dysfunction, this is acute and unlikely to result in permanent damage. However, a 10-fold larger dose, given mistakenly, resulted in death. Longer-term studies in adults have indicated no adverse consequences of moderate fluctuations in dietary methionine intake, but intakes higher than 5 times normal resulted in elevated homocysteine levels. These effects of methionine on homocysteine and vascular function are moderated by supplements of vitamins B-6, B-12, C, and folic acid. In infants, methionine intakes of 2 to 5 times normal resulted in impaired growth and extremely high plasma methionine levels, but no adverse long-term consequences were observed. (PMID 16702346 ).
Structure
Thumb
Synonyms
ValueSource
(2S)-2-amino-4-(Methylsulfanyl)butanoic acidChEBI
(S)-2-amino-4-(methylthio)Butanoic acidChEBI
(S)-2-amino-4-(methylthio)Butyric acidChEBI
(S)-MethionineChEBI
L-(-)-MethionineChEBI
L-alpha-amino-gamma-Methylmercaptobutyric acidChEBI
L-MethioninChEBI
MChEBI
MetChEBI
METHIONINEChEBI
(2S)-2-amino-4-(Methylsulfanyl)butanoateGenerator
(2S)-2-amino-4-(Methylsulphanyl)butanoateGenerator
(2S)-2-amino-4-(Methylsulphanyl)butanoic acidGenerator
(S)-2-amino-4-(methylthio)ButanoateGenerator
(S)-2-amino-4-(methylthio)ButyrateGenerator
L-a-amino-g-MethylmercaptobutyrateGenerator
L-a-amino-g-Methylmercaptobutyric acidGenerator
L-alpha-amino-gamma-MethylmercaptobutyrateGenerator
L-α-amino-γ-methylmercaptobutyrateGenerator
L-α-amino-γ-methylmercaptobutyric acidGenerator
(L)-MethionineHMDB
(S)-(+)-MethionineHMDB
(S)-2-amino-4-(methylthio)-ButanoateHMDB
(S)-2-amino-4-(methylthio)-Butanoic acidHMDB
2-amino-4-(methylthio)ButyrateHMDB
2-amino-4-(methylthio)Butyric acidHMDB
2-amino-4-MethylthiobutanoateHMDB
2-amino-4-Methylthiobutanoic acidHMDB
a-amino-g-MethylmercaptobutyrateHMDB
a-amino-g-Methylmercaptobutyric acidHMDB
AcimethinHMDB
alpha-amino-alpha-Aminobutyric acidHMDB
alpha-amino-gamma-MethylmercaptobutyrateHMDB
alpha-amino-gamma-Methylmercaptobutyric acidHMDB
CymethionHMDB
g-methylthio-a-AminobutyrateHMDB
g-methylthio-a-Aminobutyric acidHMDB
gamma-methylthio-alpha-AminobutyrateHMDB
gamma-methylthio-alpha-Aminobutyric acidHMDB
H-Met-HHMDB
H-Met-OHHMDB
L(-)-amino-alpha-amino-alpha-Aminobutyric acidHMDB
L(-)-amino-gamma-Methylthiobutyric acidHMDB
L-2-amino-4-(methylthio)Butyric acidHMDB
L-2-amino-4-Methylthiobutyric acidHMDB
L-a-amino-g-MethylthiobutyrateHMDB
L-a-amino-g-Methylthiobutyric acidHMDB
L-alpha-amino-gamma-MethylthiobutyrateHMDB
L-alpha-amino-gamma-Methylthiobutyric acidHMDB
L-gamma-methylthio-alpha-Aminobutyric acidHMDB
L-MethioninumHMDB
LiquimethHMDB
MepronHMDB
MethilaninHMDB
MethioninumHMDB
MetioninaHMDB
neo-MethidinHMDB
Poly-L-methionineHMDB
PolymethionineHMDB
S-MethionineHMDB
S-Methyl-L-homocysteineHMDB
Toxin warHMDB
Chemical FormulaC5H11NO2S
Average Molecular Weight149.211
Monoisotopic Molecular Weight149.051049291
IUPAC Name(2S)-2-amino-4-(methylsulfanyl)butanoic acid
Traditional NameL-methionine
CAS Registry Number63-68-3
SMILES
CSCC[C@H](N)C(O)=O
InChI Identifier
InChI=1S/C5H11NO2S/c1-9-3-2-4(6)5(7)8/h4H,2-3,6H2,1H3,(H,7,8)/t4-/m0/s1
InChI KeyInChIKey=FFEARJCKVFRZRR-BYPYZUCNSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as d-alpha-amino acids. These are alpha amino acids which have the D-configuration of the alpha-carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentD-alpha-amino acids
Alternative Parents
Substituents
  • D-alpha-amino acid
  • Methionine or derivatives
  • Thia fatty acid
  • Amino fatty acid
  • Fatty acyl
  • Fatty acid
  • Dialkylthioether
  • Sulfenyl compound
  • Thioether
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Hydrocarbon derivative
  • Primary amine
  • Organosulfur compound
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Drug metabolite
  • Food
Biofunction
  • Component of Aminoacyl-tRNA biosynthesis
  • Component of Glycine, serine and threonine metabolism
  • Component of Histidine metabolism
  • Component of Methionine metabolism
  • Component of Selenoamino acid metabolism
  • Component of Tyrosine metabolism
  • Essential amino acids
  • Waste products
ApplicationNot Available
Cellular locations
  • Extracellular
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point284 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility56.6 mg/mLYALKOWSKY,SH & DANNENFELSER,RM (1992)
LogP-1.87HANSCH,C ET AL. (1995)
Predicted Properties
PropertyValueSource
Water Solubility23.9 mg/mLALOGPS
logP-1.9ALOGPS
logP-2.2ChemAxon
logS-0.8ALOGPS
pKa (Strongest Acidic)2.53ChemAxon
pKa (Strongest Basic)9.5ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area63.32 Å2ChemAxon
Rotatable Bond Count4ChemAxon
Refractivity37.59 m3·mol-1ChemAxon
Polarizability15.5 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-004i-0920000000-945c85aa7c9f5eb2dfbbView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)splash10-004i-0910000000-b837ee0f4413856560f1View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-00b9-7910000000-5a1558fbb2f5e86edc9bView in MoNA
GC-MSGC-MS Spectrum - GC-MS (1 TMS)splash10-0udi-1900000000-1a97567ce4f25c4e8263View in MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-004i-0910000000-1b0477118cb20549bf4dView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MSNot Available
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0uea-1900000000-b991859b2c5bed6592a8View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-08fr-9000000000-66855ace60e59837f131View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-08fr-9000000000-56a567791c824c6a9da6View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0fe0-0900000000-d680295f21b2e2b40366View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0006-9000000000-062d3540db4db22da836View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-00di-0900000000-84467513e2c9ec1a6851View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-88dc2bebb198eea550efView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0udi-0920000000-b444ad79abeb16acde43View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-6e24a8df417e5f3db58cView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-00di-0900000000-c60ef880eb9a816a274bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-000i-0900000000-a7b7d1a3481c0c691a5bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-0002-0900000000-9156f088f4cc9eafa892View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-0002-9200000000-f78ba2aab8d5a0e0b135View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-0002-9000000000-e7b819fd2d0ac3862860View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-0002-9000000000-b61396e720381bd5ff85View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0002-9000000000-b61396e720381bd5ff85View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-0udi-0900000000-c3557cb41fd6fe268819View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-0udi-6900000000-6bf5af2d1c561013948aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-08fr-9000000000-63c9b1c138f9a27490e5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-03di-9000000000-e133c47b0efe4992589fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-03di-9000000000-dee78b4f34f8732fedb0View in MoNA
LC-MS/MSLC-MS/MS Spectrum - CE-ESI-TOF (CE-system connected to 6210 Time-of-Flight MS, Agilent) , Positivesplash10-0udi-0900000000-d3f03ff5e8eacc8b6c8eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positivesplash10-0udi-0900000000-e0dd5ff44b7962f6a2d2View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, PositiveNot Available
1D NMR13C NMR SpectrumNot Available
1D NMR1H NMR SpectrumNot Available
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,1H] 2D NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Extracellular
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Location
  • Fibroblasts
  • Kidney
  • Liver
  • Muscle
  • Pancreas
  • Prostate
  • Spleen
Pathways
NameSMPDB LinkKEGG Link
3-Phosphoglycerate dehydrogenase deficiencySMP00721Not Available
Amikacin PathwaySMP00253Not Available
Arbekacin Action PathwaySMP00713Not Available
Azithromycin PathwaySMP00247Not Available
Betaine MetabolismSMP00123map00260
Chloramphenicol Action PathwaySMP00729Not Available
Clarithromycin PathwaySMP00248Not Available
Clindamycin PathwaySMP00249Not Available
Clomocycline PathwaySMP00262Not Available
Cystathionine Beta-Synthase DeficiencySMP00177Not Available
Demeclocycline PathwaySMP00290Not Available
Dihydropyrimidine Dehydrogenase Deficiency (DHPD)SMP00179Not Available
Dimethylglycine Dehydrogenase DeficiencySMP00242Not Available
Dimethylglycine Dehydrogenase DeficiencySMP00484Not Available
Doxycycline PathwaySMP00291Not Available
Erythromycin PathwaySMP00250Not Available
Gentamicin PathwaySMP00254Not Available
Glycine and Serine MetabolismSMP00004map00260
Glycine N-methyltransferase DeficiencySMP00222Not Available
Homocystinuria-megaloblastic anemia due to defect in cobalamin metabolism, cblG complementation typeSMP00570Not Available
Hyperglycinemia, non-ketoticSMP00485Not Available
HypermethioninemiaSMP00341Not Available
Josamycin Action PathwaySMP00731Not Available
Kanamycin PathwaySMP00255Not Available
Lincomycin Action PathwaySMP00728Not Available
Lymecycline PathwaySMP00295Not Available
Methacycline Action PathwaySMP00727Not Available
Methionine Adenosyltransferase DeficiencySMP00221Not Available
Methionine MetabolismSMP00033map00270
Methylenetetrahydrofolate Reductase Deficiency (MTHFRD)SMP00340Not Available
Minocycline PathwaySMP00292Not Available
Neomycin PathwaySMP00256Not Available
Netilmicin PathwaySMP00257Not Available
Non Ketotic HyperglycinemiaSMP00223Not Available
Oxytetracycline PathwaySMP00293Not Available
Paromomycin Action PathwaySMP00714Not Available
Rolitetracycline Action PathwaySMP00726Not Available
Roxithromycin PathwaySMP00251Not Available
S-Adenosylhomocysteine (SAH) Hydrolase DeficiencySMP00214Not Available
sarcosine oncometabolite pathway SMP02313Not Available
SarcosinemiaSMP00244Not Available
Spectinomycin PathwaySMP00258Not Available
Spermidine and Spermine BiosynthesisSMP00445Not Available
Streptomycin PathwaySMP00259Not Available
Telithromycin PathwaySMP00252Not Available
Tetracycline PathwaySMP00294Not Available
Tigecycline Action PathwaySMP00712Not Available
Tobramycin Action PathwaySMP00711Not Available
Transcription/TranslationSMP00019Not Available
Troleandomycin Action PathwaySMP00730Not Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified25.0 (21.0-29.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified33.4 +/- 9 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified6-36 uMNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified4-39 uMInfant (1 - 3 months old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified35.0 +/- 5.0 uMNewborn (0-30 days old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified5-40 uMChildren (3 months - 6 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified27.0 +/- 5.0 uMChildren (1-13 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified16-37 uMChildren (6 - 18 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified32.0 +/- 6.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified27.0 +/- 5.0 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified25 +/- 6 uMAdult (>18 years old)MaleNormal details
BloodDetected and Quantified22 +/- 4 uMAdult (>18 years old)FemaleNormal details
BloodDetected and Quantified29.8 +/- 6.3 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified5 +/- 4 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified3.6 +/- 0.98 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified2.9 +/- 0.69 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified1.9 +/- 0.7 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0-5 uMChildren (0 - 10 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0-6.5 uMAdolescent (>11 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified6.1 +/- 3.1 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified4.3 +/- 2.5 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified4.07 (3.00-5.14) uMAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot ApplicableNot SpecifiedBoth
Normal
details
FecesDetected and Quantified3.01 (0–7.34) ppmInfant (0-1 year old)Not Specified
Normal
details
FecesDetected and Quantified3.52 (0–7.32) ppmInfant (0-1 year old)Not Specified
Normal
details
FecesDetected and Quantified7.72 (0–15.72) ppmInfant (0-1 year old)Not Specified
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)FemaleNormal details
FecesDetected but not QuantifiedNot ApplicableChildren (1-13 years old)Not Specified
Normal
details
FecesDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
SalivaDetected and Quantified0.0354 +/- 0.0873 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified0.176 +/- 0.152 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified2.88 +/- 5.55 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified0.418 +/- 0.420 uMAdult (>18 years old)Both
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified8.81 +/- 5.94 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
UrineDetected and Quantified1.0 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified7.134 +/- 5.264 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified0.75-1.4 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified2.303 (0.658-3.947) umol/mmol creatinineAdult (>18 years old)BothNormal
    details
    UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
    Normal
    details
    UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
    Normal
    details
    UrineDetected and Quantified1.70 +/- 1.01 umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected and Quantified0.065 (0.0-0.13) umol/mmol creatinineNewborn (0-30 days old)BothNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified0.8 (0.4-1.6) umol/mmol creatinineAdult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified0.611 +/- 0.45 umol/mmol creatinineChildren (1-13 years old)MaleNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified1.3 +/- 0.97 umol/mmol creatinineAdult (>18 years old)MaleNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified1.12 +/- 0.97 umol/mmol creatinineAdult (>18 years old)FemaleNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified1.4 (0.5-2.5) umol/mmol creatinineAdult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified0.75-1.1 umol/mmol creatinineAdult (>18 years old)MaleNormal details
    UrineDetected and Quantified0 - 7.01 umol/mmol creatinineNewborn (0-30 days old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 7.01 umol/mmol creatinineInfant (1 - 6 months old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0.4 umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected and Quantified0 - 7.01 umol/mmol creatinineInfant (6 months - <1 year old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 7.01 umol/mmol creatinineChildren (1 - 2 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 4.97 umol/mmol creatinineChildren (2 - 4 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 4.97 umol/mmol creatinineChildren (4 - 13 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 4.97 umol/mmol creatinineAdolescent (13 - 21 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 4.97 umol/mmol creatinineAdult (>21 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified3.1668 +/- 1.2788 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Normal
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified1.21 +/- 1.20 umol/mmol creatinineInfant (0-1 year old)BothNormal details
    Abnormal Concentrations
    BiofluidStatusValueAgeSexConditionReferenceDetails
    BloodDetected and Quantified17.8 (17.0-18.6) uMAdult (>18 years old)Both
    Epilepsy
    details
    BloodDetected and Quantified775.00 (250.00-1300.00) uMChildren (1-13 years old)BothMethionine adenosyl transferase deficiency details
    BloodDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
    Oesophageal cancer
    details
    BloodDetected and Quantified17.3 +/- 9.5 uMAdult (>18 years old)BothHeart Transplant details
    BloodDetected and Quantified518.5 (37.00-1000.00) uMChildren (1-13 years old)BothS-Adenosylhomocysteine hydrolase deficiency details
    BloodDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothColorectal cancer details
    BloodDetected and Quantified27 +/- 7 uMAdult (>18 years old)MaleSchizophrenia details
    BloodDetected and Quantified26 +/- 7 uMAdult (>18 years old)FemaleSchizophrenia details
    BloodDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
    Colorectal cancer
    details
    BloodDetected and Quantified750.0 (500.00-1000.00) uMChildren (1-13 years old)BothGlycine N-Methyltransferase deficiency details
    BloodDetected and Quantified20.00 (10.00-30.00) uMAdult (>18 years old)BothHomocystinuria due to defect of N(5,10)-methylene THF deficiency details
    BloodDetected and Quantified9.00 (0.00-18.00) uMChildren (1-13 years old)BothHomocystinuria details
    BloodDetected and Quantified415.0 +/- 7.0 uMAdult (>18 years old)Both
    Heart failure
    details
    Cerebrospinal Fluid (CSF)Detected and Quantified2.7 +/- 0.67 uMAdult (>18 years old)BothSchizophrenia details
    Cerebrospinal Fluid (CSF)Detected and Quantified6.0 +/- 5.3 uMChildren (1-13 years old)Not SpecifiedLeukemia details
    Cerebrospinal Fluid (CSF)Detected and Quantified3.5 +/- 0.4 uMChildren (1-13 years old)Not Specified
    Leukemia
    details
    FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
    Irritable bowel syndrome
    details
    FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
    Ulcerative colitis
    details
    FecesDetected but not QuantifiedNot ApplicableChildren (1-13 years old)Not Specified
    Treated celiac disease
    details
    UrineDetected and Quantified6.941 +/- 6.54 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothColorectal cancer details
    UrineDetected and Quantified2.1201 +/- 1.2082 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified2.0153 +/- 1.1675 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Gastroesophageal reflux disease
      • Mordechai, Hien, ...
    details
    Associated Disorders and Diseases
    Disease References
    Epilepsy
    1. Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [14992292 ]
    Schizophrenia
    1. Do KQ, Lauer CJ, Schreiber W, Zollinger M, Gutteck-Amsler U, Cuenod M, Holsboer F: gamma-Glutamylglutamine and taurine concentrations are decreased in the cerebrospinal fluid of drug-naive patients with schizophrenic disorders. J Neurochem. 1995 Dec;65(6):2652-62. [7595563 ]
    2. Bjerkenstedt L, Edman G, Hagenfeldt L, Sedvall G, Wiesel FA: Plasma amino acids in relation to cerebrospinal fluid monoamine metabolites in schizophrenic patients and healthy controls. Br J Psychiatry. 1985 Sep;147:276-82. [2415198 ]
    Heart failure
    1. Norrelund H, Wiggers H, Halbirk M, Frystyk J, Flyvbjerg A, Botker HE, Schmitz O, Jorgensen JO, Christiansen JS, Moller N: Abnormalities of whole body protein turnover, muscle metabolism and levels of metabolic hormones in patients with chronic heart failure. J Intern Med. 2006 Jul;260(1):11-21. [16789974 ]
    Homocystinuria
    1. MetaGene [Link]
    Hypermethioninemia
    1. MetaGene [Link]
    Leukemia
    1. Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. [15911239 ]
    Methionine adenosyltransferase deficiency
    1. MetaGene [Link]
    Glycine N-methyltransferase deficiency
    1. MetaGene [Link]
    Homocystinuria due to defect of N(5,10)-methylene THF deficiency
    1. MetaGene [Link]
    Associated OMIM IDs
    • 606664 (Glycine N-methyltransferase deficiency)
    • 236250 (Homocystinuria due to defect of N(5,10)-methylene THF deficiency)
    • 236200 (Homocystinuria)
    • 250850 (Methionine adenosyltransferase deficiency)
    • 613752 (Hypermethioninemia)
    • 181500 (Schizophrenia)
    DrugBank IDDB00134
    DrugBank Metabolite IDDBMET00506
    Phenol Explorer Compound IDNot Available
    Phenol Explorer Metabolite IDNot Available
    FoodDB IDFDB012683
    KNApSAcK IDC00001379
    Chemspider ID5907
    KEGG Compound IDC00073
    BioCyc IDMET
    BiGG ID33753
    Wikipedia LinkMET
    NuGOwiki LinkHMDB00696
    Metagene LinkHMDB00696
    METLIN ID5664
    PubChem Compound6137
    PDB IDMET
    ChEBI ID16643
    References
    Synthesis ReferenceBoy, Matthias; Klein, Daniela; Schroeder, Hartwig. Method for the production and recovery of methionine. PCT Int. Appl. (2005), 34 pp.
    Material Safety Data Sheet (MSDS)Download (PDF)
    General References
    1. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. [19212411 ]
    2. Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. [2026685 ]
    3. Engelborghs S, Marescau B, De Deyn PP: Amino acids and biogenic amines in cerebrospinal fluid of patients with Parkinson's disease. Neurochem Res. 2003 Aug;28(8):1145-50. [12834252 ]
    4. Hagenfeldt L, Bjerkenstedt L, Edman G, Sedvall G, Wiesel FA: Amino acids in plasma and CSF and monoamine metabolites in CSF: interrelationship in healthy subjects. J Neurochem. 1984 Mar;42(3):833-7. [6198473 ]
    5. Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. [15911239 ]
    6. Cynober LA: Plasma amino acid levels with a note on membrane transport: characteristics, regulation, and metabolic significance. Nutrition. 2002 Sep;18(9):761-6. [12297216 ]
    7. Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [14992292 ]
    8. Kersemans V, Cornelissen B, Kersemans K, Bauwens M, Achten E, Dierckx RA, Mertens J, Slegers G: In vivo characterization of 123/125I-2-iodo-L-phenylalanine in an R1M rhabdomyosarcoma athymic mouse model as a potential tumor tracer for SPECT. J Nucl Med. 2005 Mar;46(3):532-9. [15750170 ]
    9. Alme B, Bremmelgaard A, Sjovall J, Thomassen P: Analysis of metabolic profiles of bile acids in urine using a lipophilic anion exchanger and computerized gas-liquid chromatorgaphy-mass spectrometry. J Lipid Res. 1977 May;18(3):339-62. [864325 ]
    10. Sardharwalla IB, Fowler B, Robins AJ, Komrower GM: Detection of heterozygotes for homocystinuria. Study of sulphur-containing amino acids in plasma and urine after L-methionine loading. Arch Dis Child. 1974 Jul;49(7):553-9. [4851308 ]
    11. Alton KB, Hernandez A, Alvarez N, Patrick JE: High-performance liquid chromatographic determination of N-[2(S)-(mercaptomethyl)-3-(2-methylphenyl)-1-oxopropyl]-L-methionine, the active plasma metabolite of a prodrug atriopeptidase inhibitor (SCH 42495), using a thiol selective (Au/Hg) amperometric detector. J Chromatogr. 1992 Sep 2;579(2):307-17. [1429978 ]
    12. Fischer JL, Lancia JK, Mathur A, Smith ML: Selenium protection from DNA damage involves a Ref1/p53/Brca1 protein complex. Anticancer Res. 2006 Mar-Apr;26(2A):899-904. [16619485 ]
    13. Ditscheid B, Funfstuck R, Busch M, Schubert R, Gerth J, Jahreis G: Effect of L-methionine supplementation on plasma homocysteine and other free amino acids: a placebo-controlled double-blind cross-over study. Eur J Clin Nutr. 2005 Jun;59(6):768-75. [15870821 ]
    14. Hesse A, Heimbach D: Causes of phosphate stone formation and the importance of metaphylaxis by urinary acidification: a review. World J Urol. 1999 Oct;17(5):308-15. [10552150 ]
    15. Harth G, Horwitz MA: Inhibition of Mycobacterium tuberculosis glutamine synthetase as a novel antibiotic strategy against tuberculosis: demonstration of efficacy in vivo. Infect Immun. 2003 Jan;71(1):456-64. [12496196 ]
    16. Takasu A, Shimosegawa T, Shimosegawa E, Hatazawa J, Kimura K, Fujita M, Koizumi M, Kanno I, Toyota T: 11C-methionine uptake to the pancreas and its secretion: a positron emission tomography study in humans. Pancreas. 1999 May;18(4):392-8. [10231845 ]
    17. Ball RO, Courtney-Martin G, Pencharz PB: The in vivo sparing of methionine by cysteine in sulfur amino acid requirements in animal models and adult humans. J Nutr. 2006 Jun;136(6 Suppl):1682S-1693S. [16702340 ]
    18. van de Poll MC, Dejong CH, Soeters PB: Adequate range for sulfur-containing amino acids and biomarkers for their excess: lessons from enteral and parenteral nutrition. J Nutr. 2006 Jun;136(6 Suppl):1694S-1700S. [16702341 ]
    19. Garlick PJ: Toxicity of methionine in humans. J Nutr. 2006 Jun;136(6 Suppl):1722S-1725S. [16702346 ]

    Enzymes

    General function:
    Involved in cobalamin binding
    Specific function:
    Catalyzes the transfer of a methyl group from methyl-cobalamin to homocysteine, yielding enzyme-bound cob(I)alamin and methionine. Subsequently, remethylates the cofactor using methyltetrahydrofolate (By similarity).
    Gene Name:
    MTR
    Uniprot ID:
    Q99707
    Molecular weight:
    140525.91
    Reactions
    5-Methyltetrahydrofolic acid + Homocysteine → Tetrahydrofolic acid + L-Methioninedetails
    References
    1. Taurog RE, Matthews RG: Activation of methyltetrahydrofolate by cobalamin-independent methionine synthase. Biochemistry. 2006 Apr 25;45(16):5092-102. [16618098 ]
    2. Hughes JA: In vivo hydrolysis of S-adenosyl-L-methionine in Escherichia coli increases export of 5-methylthioribose. Can J Microbiol. 2006 Jun;52(6):599-602. [16788729 ]
    3. Reynolds E: Vitamin B12, folic acid, and the nervous system. Lancet Neurol. 2006 Nov;5(11):949-60. [17052662 ]
    4. Banks EC, Doughty SW, Toms SM, Wheelhouse RT, Nicolaou A: Inhibition of cobalamin-dependent methionine synthase by substituted benzo-fused heterocycles. FEBS J. 2007 Jan;274(1):287-99. [17222188 ]
    5. Yamada K, Kawata T, Wada M, Mori K, Tamai H, Tanaka N, Tadokoro T, Tobimatsu T, Toraya T, Maekawa A: Testicular injury to rats fed on soybean protein-based vitamin B12-deficient diet can be reduced by methionine supplementation. J Nutr Sci Vitaminol (Tokyo). 2007 Apr;53(2):95-101. [17615995 ]
    General function:
    Involved in methylenetetrahydrofolate reductase (NADPH) activity
    Specific function:
    Catalyzes the conversion of 5,10-methylenetetrahydrofolate to 5-methyltetrahydrofolate, a co-substrate for homocysteine remethylation to methionine.
    Gene Name:
    MTHFR
    Uniprot ID:
    P42898
    Molecular weight:
    74595.895
    References
    1. Lor KL, Cossins EA: Regulation of C metabolism by L-methionine in Saccharomyces cerevisiae. Biochem J. 1972 Dec;130(3):773-83. [4198357 ]
    General function:
    Involved in 1-aminocyclopropane-1-carboxylate synthase activity
    Specific function:
    Transaminase involved in tyrosine breakdown. Converts tyrosine to p-hydroxyphenylpyruvate. Can catalyze the reverse reaction, using glutamic acid, with 2-oxoglutarate as cosubstrate (in vitro). Has much lower affinity and transaminase activity towards phenylalanine.
    Gene Name:
    TAT
    Uniprot ID:
    P17735
    Molecular weight:
    50398.895
    Reactions
    2-Oxo-4-methylthiobutanoic acid + L-Glutamic acid → L-Methionine + Oxoglutaric aciddetails
    General function:
    Involved in methionine adenosyltransferase activity
    Specific function:
    Catalyzes the formation of S-adenosylmethionine from methionine and ATP.
    Gene Name:
    MAT2A
    Uniprot ID:
    P31153
    Molecular weight:
    43660.37
    Reactions
    Adenosine triphosphate + L-Methionine + Water → Phosphoric acid + Pyrophosphate + S-Adenosylmethioninedetails
    Phosphoric acid + Pyrophosphate + S-Adenosylmethionine → Adenosine triphosphate + L-Methionine + Waterdetails
    References
    1. Rodriguez JL, Boukaba A, Sandoval J, Georgieva EI, Latasa MU, Garcia-Trevijano ER, Serviddio G, Nakamura T, Avila MA, Sastre J, Torres L, Mato JM, Lopez-Rodas G: Transcription of the MAT2A gene, coding for methionine adenosyltransferase, is up-regulated by E2F and Sp1 at a chromatin level during proliferation of liver cells. Int J Biochem Cell Biol. 2007;39(4):842-50. Epub 2007 Jan 20. [17317269 ]
    2. Yang H, Magilnick N, Noureddin M, Mato JM, Lu SC: Effect of hepatocyte growth factor on methionine adenosyltransferase genes and growth is cell density-dependent in HepG2 cells. J Cell Physiol. 2007 Mar;210(3):766-73. [17154373 ]
    3. Lin WC, Lin WL: Ameliorative effect of Ganoderma lucidum on carbon tetrachloride-induced liver fibrosis in rats. World J Gastroenterol. 2006 Jan 14;12(2):265-70. [16482628 ]
    4. Chen H, Xia M, Lin M, Yang H, Kuhlenkamp J, Li T, Sodir NM, Chen YH, Josef-Lenz H, Laird PW, Clarke S, Mato JM, Lu SC: Role of methionine adenosyltransferase 2A and S-adenosylmethionine in mitogen-induced growth of human colon cancer cells. Gastroenterology. 2007 Jul;133(1):207-18. Epub 2007 Apr 11. [17631143 ]
    General function:
    Involved in methyltransferase activity
    Specific function:
    Catalyzes the transfer of a methyl group from AdoMet to trivalent arsenicals producing methylated and dimethylated arsenicals. It methylates arsenite to form methylarsonate, Me-AsO(3)H(2), which is reduced by methylarsonate reductase to methylarsonite, Me-As(OH)2. Methylarsonite is also a substrate and it is converted into the much less toxic compound dimethylarsinate (cacodylate), Me(2)As(O)-OH (By similarity).
    Gene Name:
    AS3MT
    Uniprot ID:
    Q9HBK9
    Molecular weight:
    41747.49
    General function:
    Involved in methyltransferase activity
    Specific function:
    Functions as thioether S-methyltransferase and is active with a variety of thioethers and the corresponding selenium and tellurium compounds, including 3-methylthiopropionaldehyde, dimethyl selenide, dimethyl telluride, 2-methylthioethylamine, 2-methylthioethanol, methyl-n-propyl sulfide and diethyl sulfide. Plays an important role in the detoxification of selenium compounds (By similarity). Catalyzes the N-methylation of tryptamine and structurally related compounds.
    Gene Name:
    INMT
    Uniprot ID:
    O95050
    Molecular weight:
    28833.695
    General function:
    Involved in methionine adenosyltransferase activity
    Specific function:
    Catalyzes the formation of S-adenosylmethionine from methionine and ATP.
    Gene Name:
    MAT1A
    Uniprot ID:
    Q00266
    Molecular weight:
    43647.6
    Reactions
    Adenosine triphosphate + L-Methionine + Water → Phosphoric acid + Pyrophosphate + S-Adenosylmethioninedetails
    Phosphoric acid + Pyrophosphate + S-Adenosylmethionine → Adenosine triphosphate + L-Methionine + Waterdetails
    References
    1. Linnebank M, Lagler F, Muntau AC, Roschinger W, Olgemoller B, Fowler B, Koch HG: Methionine adenosyltransferase (MAT) I/III deficiency with concurrent hyperhomocysteinaemia: two novel cases. J Inherit Metab Dis. 2005;28(6):1167-8. [16435220 ]
    2. Yang H, Magilnick N, Noureddin M, Mato JM, Lu SC: Effect of hepatocyte growth factor on methionine adenosyltransferase genes and growth is cell density-dependent in HepG2 cells. J Cell Physiol. 2007 Mar;210(3):766-73. [17154373 ]
    3. Chen H, Xia M, Lin M, Yang H, Kuhlenkamp J, Li T, Sodir NM, Chen YH, Josef-Lenz H, Laird PW, Clarke S, Mato JM, Lu SC: Role of methionine adenosyltransferase 2A and S-adenosylmethionine in mitogen-induced growth of human colon cancer cells. Gastroenterology. 2007 Jul;133(1):207-18. Epub 2007 Apr 11. [17631143 ]
    4. Rodriguez JL, Boukaba A, Sandoval J, Georgieva EI, Latasa MU, Garcia-Trevijano ER, Serviddio G, Nakamura T, Avila MA, Sastre J, Torres L, Mato JM, Lopez-Rodas G: Transcription of the MAT2A gene, coding for methionine adenosyltransferase, is up-regulated by E2F and Sp1 at a chromatin level during proliferation of liver cells. Int J Biochem Cell Biol. 2007;39(4):842-50. Epub 2007 Jan 20. [17317269 ]
    5. Prudova A, Bauman Z, Braun A, Vitvitsky V, Lu SC, Banerjee R: S-adenosylmethionine stabilizes cystathionine beta-synthase and modulates redox capacity. Proc Natl Acad Sci U S A. 2006 Apr 25;103(17):6489-94. Epub 2006 Apr 13. [16614071 ]
    General function:
    Involved in oxidoreductase activity
    Specific function:
    Involved in the reductive regeneration of cob(I)alamin cofactor required for the maintenance of methionine synthase in a functional state.
    Gene Name:
    MTRR
    Uniprot ID:
    Q9UBK8
    Molecular weight:
    77672.995
    References
    1. Kim DJ, Park BL, Koh JM, Kim GS, Kim LH, Cheong HS, Shin HD, Hong JM, Kim TH, Shin HI, Park EK, Kim SY: Methionine synthase reductase polymorphisms are associated with serum osteocalcin levels in postmenopausal women. Exp Mol Med. 2006 Oct 31;38(5):519-24. [17079868 ]
    2. Tvedegaard KC, Rudiger NS, Pedersen BN, Moller J: Detection of MTRR 66A-->G polymorphism using the real-time polymerase chain reaction machine LightCycler for determination of composition of allele after restriction cleavage. Scand J Clin Lab Invest. 2006;66(8):685-93. [17101561 ]
    3. Elmore CL, Wu X, Leclerc D, Watson ED, Bottiglieri T, Krupenko NI, Krupenko SA, Cross JC, Rozen R, Gravel RA, Matthews RG: Metabolic derangement of methionine and folate metabolism in mice deficient in methionine synthase reductase. Mol Genet Metab. 2007 May;91(1):85-97. Epub 2007 Mar 21. [17369066 ]
    General function:
    Involved in 1-aminocyclopropane-1-carboxylate synthase activity
    Specific function:
    Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). Metabolizes the cysteine conjugates of certain halogenated alkenes and alkanes to form reactive metabolites. Catalyzes the beta-elimination of S-conjugates and Se-conjugates of L-(seleno)cysteine, resulting in the cleavage of the C-S or C-Se bond.
    Gene Name:
    CCBL1
    Uniprot ID:
    Q16773
    Molecular weight:
    47874.765
    Reactions
    Se-Methylselenocysteine + 2-Oxo-4-methylthiobutanoic acid → Methylselenopyruvate + L-Methioninedetails
    General function:
    Involved in oxidoreductase activity, acting on a sulfur group of donors, disulfide as acceptor
    Specific function:
    Has an important function as a repair enzyme for proteins that have been inactivated by oxidation. Catalyzes the reversible oxidation-reduction of methionine sulfoxide in proteins to methionine.
    Gene Name:
    MSRA
    Uniprot ID:
    Q9UJ68
    Molecular weight:
    21737.41
    Reactions
    L-Methionine + thioredoxin disulfide + Water → Methionine sulfoxide + thioredoxindetails
    References
    1. Schallreuter KU, Rubsam K, Chavan B, Zothner C, Gillbro JM, Spencer JD, Wood JM: Functioning methionine sulfoxide reductases A and B are present in human epidermal melanocytes in the cytosol and in the nucleus. Biochem Biophys Res Commun. 2006 Mar 31;342(1):145-52. Epub 2006 Feb 3. [16480945 ]
    2. Su Z, Limberis J, Martin RL, Xu R, Kolbe K, Heinemann SH, Hoshi T, Cox BF, Gintant GA: Functional consequences of methionine oxidation of hERG potassium channels. Biochem Pharmacol. 2007 Sep 1;74(5):702-11. Epub 2007 Jun 7. [17624316 ]
    3. Oien DB, Moskovitz J: Ablation of the mammalian methionine sulfoxide reductase A affects the expression level of cysteine deoxygenase. Biochem Biophys Res Commun. 2007 Jan 12;352(2):556-9. Epub 2006 Nov 20. [17126812 ]
    4. Rouhier N, Vieira Dos Santos C, Tarrago L, Rey P: Plant methionine sulfoxide reductase A and B multigenic families. Photosynth Res. 2006 Sep;89(2-3):247-62. Epub 2006 Sep 22. [17031545 ]
    5. Gand A, Antoine M, Boschi-Muller S, Branlant G: Characterization of the amino acids involved in substrate specificity of methionine sulfoxide reductase A. J Biol Chem. 2007 Jul 13;282(28):20484-91. Epub 2007 May 11. [17500063 ]
    General function:
    Involved in zinc ion binding
    Specific function:
    Involved in the regulation of homocysteine metabolism. Converts betaine and homocysteine to dimethylglycine and methionine, respectively. This reaction is also required for the irreversible oxidation of choline.
    Gene Name:
    BHMT
    Uniprot ID:
    Q93088
    Molecular weight:
    44998.205
    Reactions
    Trimethylammonioacetate + Homocysteine → Dimethylglycine + L-Methioninedetails
    References
    1. Slow S, Garrow TA: Liver choline dehydrogenase and kidney betaine-homocysteine methyltransferase expression are not affected by methionine or choline intake in growing rats. J Nutr. 2006 Sep;136(9):2279-83. [16920841 ]
    2. Sparks JD, Collins HL, Chirieac DV, Cianci J, Jokinen J, Sowden MP, Galloway CA, Sparks CE: Hepatic very-low-density lipoprotein and apolipoprotein B production are increased following in vivo induction of betaine-homocysteine S-methyltransferase. Biochem J. 2006 Apr 15;395(2):363-71. [16396637 ]
    3. Uthus EO, Reeves PG, Saari JT: Copper deficiency decreases plasma homocysteine in rats. J Nutr. 2007 Jun;137(6):1370-4. [17513393 ]
    4. Liu J, Xie Y, Cooper R, Ducharme DM, Tennant R, Diwan BA, Waalkes MP: Transplacental exposure to inorganic arsenic at a hepatocarcinogenic dose induces fetal gene expression changes in mice indicative of aberrant estrogen signaling and disrupted steroid metabolism. Toxicol Appl Pharmacol. 2007 May 1;220(3):284-91. Epub 2007 Feb 6. [17350061 ]
    5. Hazra A, Wu K, Kraft P, Fuchs CS, Giovannucci EL, Hunter DJ: Twenty-four non-synonymous polymorphisms in the one-carbon metabolic pathway and risk of colorectal adenoma in the Nurses' Health Study. Carcinogenesis. 2007 Jul;28(7):1510-9. Epub 2007 Mar 26. [17389618 ]
    General function:
    Involved in nucleotide binding
    Specific function:
    Not Available
    Gene Name:
    MARS
    Uniprot ID:
    P56192
    Molecular weight:
    101114.925
    Reactions
    Adenosine triphosphate + L-Methionine + tRNA(Met) → Adenosine monophosphate + Pyrophosphate + L-methionyl-tRNA(Met)details
    Adenosine triphosphate + L-Methionine + tRNA(Met) → Adenosine monophosphate + Pyrophosphate + L-Methionyl-tRNAdetails
    References
    1. Chwatko G, Boers GH, Strauss KA, Shih DM, Jakubowski H: Mutations in methylenetetrahydrofolate reductase or cystathionine beta-synthase gene, or a high-methionine diet, increase homocysteine thiolactone levels in humans and mice. FASEB J. 2007 Jun;21(8):1707-13. Epub 2007 Feb 27. [17327360 ]
    2. Jakubowski H: Pathophysiological consequences of homocysteine excess. J Nutr. 2006 Jun;136(6 Suppl):1741S-1749S. [16702349 ]
    3. Vaughan MD, Sampson PB, Daub E, Honek JF: Investigation of bioisosteric effects on the interaction of substrates/ inhibitors with the methionyl-tRNA synthetase from Escherichia coli. Med Chem. 2005 May;1(3):227-37. [16787318 ]
    General function:
    Involved in glutamate-ammonia ligase activity
    Specific function:
    This enzyme has 2 functions: it catalyzes the production of glutamine and 4-aminobutanoate (gamma-aminobutyric acid, GABA), the latter in a pyridoxal phosphate-independent manner (By similarity). Essential for proliferation of fetal skin fibroblasts.
    Gene Name:
    GLUL
    Uniprot ID:
    P15104
    Molecular weight:
    42064.15
    References
    1. Rowe WB, Meister A: Studies on the inhibition of glutamine synthetase by methionine sulfone. Biochemistry. 1973 Apr 10;12(8):1578-82. [4144717 ]
    2. Rowe WB, Ronzio RA, Meister A: Inhibition of glutamine synthetase by methionine sulfoximine. Studies on methionine sulfoximine phosphate. Biochemistry. 1969 Jun;8(6):2674-80. [4308004 ]
    3. Orr J, Haselkorn R: Regulation of glutamine synthetase activity and synthesis in free-living and symbiotic Anabaena spp. J Bacteriol. 1982 Nov;152(2):626-35. [6127334 ]
    General function:
    Involved in catalytic activity
    Specific function:
    Catalyzes the radical-mediated insertion of two sulfur atoms into the C-6 and C-8 positions of the octanoyl moiety bound to the lipoyl domains of lipoate-dependent enzymes, thereby converting the octanoylated domains into lipoylated derivatives (By similarity).
    Gene Name:
    LIAS
    Uniprot ID:
    O43766
    Molecular weight:
    41910.695
    Reactions
    Protein N(6)-(octanoyl)lysine + Hydrogen sulfide + S-Adenosylmethionine → protein N(6)-(lipoyl)lysine + L-Methionine + 5'-Deoxyadenosinedetails
    Protein N6-(octanoyl)lysine + Sulfur donor + S-Adenosylmethionine → Protein N6-(lipoyl)lysine + L-Methionine + 5'-Deoxyadenosinedetails
    Octanoyl-[acp] + Sulfur donor + S-Adenosylmethionine → Lipoyl-[acp] + L-Methionine + 5'-Deoxyadenosinedetails
    General function:
    Involved in dTDP-4-dehydrorhamnose reductase activity
    Specific function:
    Non-catalytic regulatory subunit of S-adenosylmethionine synthetase 2 (MAT2A), an enzyme that catalyzes the formation of S-adenosylmethionine from methionine and ATP. Regulates the activity of S-adenosylmethionine synthetase 2 by changing its kinetic properties, rendering the enzyme more susceptible to S-adenosylmethionine inhibition.
    Gene Name:
    MAT2B
    Uniprot ID:
    Q9NZL9
    Molecular weight:
    Not Available
    Reactions
    Phosphoric acid + Pyrophosphate + S-Adenosylmethionine → Adenosine triphosphate + L-Methionine + Waterdetails
    References
    1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [17139284 ]
    2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [17016423 ]
    3. LeGros L, Halim AB, Chamberlin ME, Geller A, Kotb M: Regulation of the human MAT2B gene encoding the regulatory beta subunit of methionine adenosyltransferase, MAT II. J Biol Chem. 2001 Jul 6;276(27):24918-24. Epub 2001 May 3. [11337507 ]
    General function:
    Involved in zinc ion binding
    Specific function:
    Involved in the regulation of homocysteine metabolism. Converts homocysteine to methionine using S-methylmethionine (SMM) as a methyl donor.
    Gene Name:
    BHMT2
    Uniprot ID:
    Q9H2M3
    Molecular weight:
    33166.69
    Reactions
    Trimethylammonioacetate + Homocysteine → Dimethylglycine + L-Methioninedetails
    References
    1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [17139284 ]
    2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [17016423 ]
    General function:
    Involved in aminopeptidase activity
    Specific function:
    Removes the amino-terminal methionine from nascent proteins. The catalytic activity of human METAP2 toward Met-Val peptides is consistently two orders of magnitude higher than that of METAP1, suggesting that it is responsible for processing proteins containing N-terminal Met-Val and Met-Thr sequences in vivo
    Gene Name:
    METAP2
    Uniprot ID:
    P50579
    Molecular weight:
    52891.1
    References
    1. Upadhya R, Zhang HS, Weiss LM: System for expression of microsporidian methionine amino peptidase type 2 (MetAP2) in the yeast Saccharomyces cerevisiae. Antimicrob Agents Chemother. 2006 Oct;50(10):3389-95. Epub 2006 Aug 17. [16917013 ]
    2. Sheppard GS, Wang J, Kawai M, Fidanze SD, BaMaung NY, Erickson SA, Barnes DM, Tedrow JS, Kolaczkowski L, Vasudevan A, Park DC, Wang GT, Sanders WJ, Mantei RA, Palazzo F, Tucker-Garcia L, Lou P, Zhang Q, Park CH, Kim KH, Petros A, Olejniczak E, Nettesheim D, Hajduk P, Henkin J, Lesniewski R, Davidsen SK, Bell RL: Discovery and optimization of anthranilic acid sulfonamides as inhibitors of methionine aminopeptidase-2: a structural basis for the reduction of albumin binding. J Med Chem. 2006 Jun 29;49(13):3832-49. [16789740 ]
    3. Addlagatta A, Matthews BW: Structure of the angiogenesis inhibitor ovalicin bound to its noncognate target, human Type 1 methionine aminopeptidase. Protein Sci. 2006 Aug;15(8):1842-8. Epub 2006 Jul 5. [16823043 ]
    4. Hu X, Addlagatta A, Lu J, Matthews BW, Liu JO: Elucidation of the function of type 1 human methionine aminopeptidase during cell cycle progression. Proc Natl Acad Sci U S A. 2006 Nov 28;103(48):18148-53. Epub 2006 Nov 17. [17114291 ]
    5. Nonato MC, Widom J, Clardy J: Human methionine aminopeptidase type 2 in complex with L- and D-methionine. Bioorg Med Chem Lett. 2006 May 15;16(10):2580-3. Epub 2006 Mar 15. [16540317 ]
    General function:
    Involved in oxidoreductase activity
    Specific function:
    Lysosomal L-amino-acid oxidase with highest specific activity with phenylalanine. May play a role in lysosomal antigen processing and presentation (By similarity).
    Gene Name:
    IL4I1
    Uniprot ID:
    Q96RQ9
    Molecular weight:
    65327.26
    Reactions
    L-Methionine + Water + Oxygen → 2-Oxo-4-methylthiobutanoic acid + Ammonia + Hydrogen peroxidedetails
    General function:
    Involved in transferase activity, transferring nitrogenous groups
    Specific function:
    Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). May catalyze the beta-elimination of S-conjugates and Se-conjugates of L-(seleno)cysteine, resulting in the cleavage of the C-S or C-Se bond (By similarity). Has transaminase activity towards L-kynurenine, tryptophan, phenylalanine, serine, cysteine, methionine, histidine, glutamine and asparagine with glyoxylate as an amino group acceptor (in vitro). Has lower activity with 2-oxoglutarate as amino group acceptor (in vitro) (By similarity).
    Gene Name:
    CCBL2
    Uniprot ID:
    Q6YP21
    Molecular weight:
    51399.855
    Reactions
    Se-Methylselenocysteine + 2-Oxo-4-methylthiobutanoic acid → Methylselenopyruvate + L-Methioninedetails
    General function:
    Involved in peptide-methionine-(S)-S-oxide reductase activity
    Specific function:
    Catalyzes the reduction of free and protein-bound methionine sulfoxide to methionine (By similarity). Upon oxidative stress, may play a role in the preservation of mitochondrial integrity by decreasing the intracellular reactive oxygen species build-up through its scavenging role, hence contributing to cell survival and protein maintenance.
    Gene Name:
    MSRB2
    Uniprot ID:
    Q9Y3D2
    Molecular weight:
    19536.055
    Reactions
    L-Methionine + oxidized thioredoxin → L-methionine R-oxide + reduced thioredoxindetails
    References
    1. Schallreuter KU, Rubsam K, Chavan B, Zothner C, Gillbro JM, Spencer JD, Wood JM: Functioning methionine sulfoxide reductases A and B are present in human epidermal melanocytes in the cytosol and in the nucleus. Biochem Biophys Res Commun. 2006 Mar 31;342(1):145-52. Epub 2006 Feb 3. [16480945 ]
    2. Ranaivoson FM, Kauffmann B, Neiers F, Wu J, Boschi-Muller S, Panjikar S, Aubry A, Branlant G, Favier F: The X-ray structure of the N-terminal domain of PILB from Neisseria meningitidis reveals a thioredoxin-fold. J Mol Biol. 2006 Apr 28;358(2):443-54. Epub 2006 Feb 28. [16530221 ]
    General function:
    Involved in peptide-methionine-(S)-S-oxide reductase activity
    Specific function:
    Catalyzes the reduction of free and protein-bound methionine sulfoxide to methionine. Isoform 2 is essential for hearing.
    Gene Name:
    MSRB3
    Uniprot ID:
    Q8IXL7
    Molecular weight:
    20010.23
    Reactions
    L-Methionine + oxidized thioredoxin → L-methionine R-oxide + reduced thioredoxindetails
    General function:
    Involved in nucleotide binding
    Specific function:
    Not Available
    Gene Name:
    MARS2
    Uniprot ID:
    Q96GW9
    Molecular weight:
    66590.29
    Reactions
    Adenosine triphosphate + L-Methionine + tRNA(Met) → Adenosine monophosphate + Pyrophosphate + L-methionyl-tRNA(Met)details
    Adenosine triphosphate + L-Methionine + tRNA(Met) → Adenosine monophosphate + Pyrophosphate + L-Methionyl-tRNAdetails
    References
    1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [17139284 ]
    2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [17016423 ]
    3. Spencer AC, Heck A, Takeuchi N, Watanabe K, Spremulli LL: Characterization of the human mitochondrial methionyl-tRNA synthetase. Biochemistry. 2004 Aug 3;43(30):9743-54. [15274629 ]
    General function:
    Involved in peptide-methionine-(S)-S-oxide reductase activity
    Specific function:
    Not Available
    Gene Name:
    MSRB1
    Uniprot ID:
    Q9NZV6
    Molecular weight:
    12760.095
    Reactions
    L-Methionine + oxidized thioredoxin → L-methionine R-oxide + reduced thioredoxindetails
    References
    1. Kim HY, Gladyshev VN: Alternative first exon splicing regulates subcellular distribution of methionine sulfoxide reductases. BMC Mol Biol. 2006 Mar 16;7:11. [16542431 ]
    2. Kim HY, Fomenko DE, Yoon YE, Gladyshev VN: Catalytic advantages provided by selenocysteine in methionine-S-sulfoxide reductases. Biochemistry. 2006 Nov 21;45(46):13697-704. [17105189 ]

    Transporters

    General function:
    Involved in transmembrane transport
    Specific function:
    Sodium-independent transporter that mediates the update of aromatic acid. Can function as a net efflux pathway for aromatic amino acids in the basosolateral epithelial cells
    Gene Name:
    SLC16A10
    Uniprot ID:
    Q8TF71
    Molecular weight:
    55492.1
    References
    1. Kim DK, Kanai Y, Chairoungdua A, Matsuo H, Cha SH, Endou H: Expression cloning of a Na+-independent aromatic amino acid transporter with structural similarity to H+/monocarboxylate transporters. J Biol Chem. 2001 May 18;276(20):17221-8. Epub 2001 Feb 20. [11278508 ]