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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-05-20 20:40:44 UTC
HMDB IDHMDB0000148
Secondary Accession Numbers
  • HMDB00148
Metabolite Identification
Common NameL-Glutamic acid
DescriptionGlutamic acid (Glu), also referred to as glutamate (the anion), is one of the 20 proteinogenic amino acids. It is not among the essential amino acids. Glutamate is a key molecule in cellular metabolism. In humans, dietary proteins are broken down by digestion into amino acids, which serves as metabolic fuel or other functional roles in the body. Glutamate is the most abundant fast excitatory neurotransmitter in the mammalian nervous system. At chemical synapses, glutamate is stored in vesicles. Nerve impulses trigger release of glutamate from the pre-synaptic cell. In the opposing post-synaptic cell, glutamate receptors, such as the NMDA receptor, bind glutamate and are activated. Because of its role in synaptic plasticity, it is believed that glutamic acid is involved in cognitive functions like learning and memory in the brain. Glutamate transporters are found in neuronal and glial membranes. They rapidly remove glutamate from the extracellular space. In brain injury or disease, they can work in reverse and excess glutamate can accumulate outside cells. This process causes calcium ions to enter cells via NMDA receptor channels, leading to neuronal damage and eventual cell death, and is called excitotoxicity. The mechanisms of cell death include: Damage to mitochondria from excessively high intracellular Ca2+. Glu/Ca2+-mediated promotion of transcription factors for pro-apoptotic genes, or downregulation of transcription factors for anti-apoptotic genes. Excitotoxicity due to glutamate occurs as part of the ischemic cascade and is associated with stroke and diseases like amyotrophic lateral sclerosis, lathyrism, and Alzheimer's disease. glutamic acid has been implicated in epileptic seizures. Microinjection of glutamic acid into neurons produces spontaneous depolarization around one second apart, and this firing pattern is similar to what is known as paroxysmal depolarizing shift in epileptic attacks. This change in the resting membrane potential at seizure foci could cause spontaneous opening of voltage activated calcium channels, leading to glutamic acid release and further depolarization (http://en.wikipedia.org/wiki/Glutamic_acid). Moreover, glutamic acid is found to be associated with N-acetylglutamate synthetase deficiency, which is an inborn error of metabolism.
Structure
Thumb
Synonyms
ValueSource
(S)-2-Aminopentanedioic acidChEBI
(S)-Glutamic acidChEBI
Acide glutamiqueChEBI
acido GlutamicoChEBI
Acidum glutamicumChEBI
EChEBI
GluChEBI
GlutamateChEBI
GLUTAMIC ACIDChEBI
L-GluChEBI
L-Glutaminic acidChEBI
L-GlutaminsaeureChEBI
(S)-2-AminopentanedioateGenerator
L-GlutamateGenerator
(S)-GlutamateGenerator
L-GlutaminateGenerator
(2S)-2-AminopentanedioateHMDB
(2S)-2-Aminopentanedioic acidHMDB
(S)-(+)-GlutamateHMDB
(S)-(+)-Glutamic acidHMDB
1-amino-Propane-1,3-dicarboxylateHMDB
1-amino-Propane-1,3-dicarboxylic acidHMDB
1-Aminopropane-1,3-dicarboxylateHMDB
1-Aminopropane-1,3-dicarboxylic acidHMDB
2-AminoglutarateHMDB
2-Aminoglutaric acidHMDB
2-AminopentanedioateHMDB
2-Aminopentanedioic acidHMDB
a-AminoglutarateHMDB
a-Aminoglutaric acidHMDB
a-GlutamateHMDB
a-Glutamic acidHMDB
AciglutHMDB
alpha-AminoglutarateHMDB
alpha-Aminoglutaric acidHMDB
alpha-GlutamateHMDB
alpha-Glutamic acidHMDB
AminoglutarateHMDB
Aminoglutaric acidHMDB
GltHMDB
GlusateHMDB
GlutHMDB
GlutacidHMDB
GlutamicolHMDB
GlutamidexHMDB
GlutaminateHMDB
Glutaminic acidHMDB
GlutaminolHMDB
GlutatonHMDB
L-(+)-GlutamateHMDB
L-(+)-Glutamic acidHMDB
L-a-AminoglutarateHMDB
L-a-Aminoglutaric acidHMDB
L-alpha-AminoglutarateHMDB
L-alpha-Aminoglutaric acidHMDB
D GlutamateMeSH
Glutamate, potassiumMeSH
Glutamic acid, (D)-isomerMeSH
L Glutamic acidMeSH
D-GlutamateMeSH
L GlutamateMeSH
Aluminum L glutamateMeSH
Aluminum L-glutamateMeSH
L-Glutamate, aluminumMeSH
Potassium glutamateMeSH
Chemical FormulaC5H9NO4
Average Molecular Weight147.1293
Monoisotopic Molecular Weight147.053157781
IUPAC Name(2S)-2-aminopentanedioic acid
Traditional NameL-glutamic acid
CAS Registry Number56-86-0
SMILES
N[C@@H](CCC(O)=O)C(O)=O
InChI Identifier
InChI=1S/C5H9NO4/c6-3(5(9)10)1-2-4(7)8/h3H,1-2,6H2,(H,7,8)(H,9,10)/t3-/m0/s1
InChI KeyWHUUTDBJXJRKMK-VKHMYHEASA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as glutamic acid and derivatives. These are compounds containing glutamic acid or a derivative thereof resulting from reaction of glutamic acid at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentGlutamic acid and derivatives
Alternative Parents
Substituents
  • Glutamic acid or derivatives
  • Alpha-amino acid
  • L-alpha-amino acid
  • Amino fatty acid
  • Dicarboxylic acid or derivatives
  • Fatty acid
  • Fatty acyl
  • Amino acid
  • Carboxylic acid
  • Organic oxide
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Organopnictogen compound
  • Carbonyl group
  • Organic oxygen compound
  • Amine
  • Organic nitrogen compound
  • Hydrocarbon derivative
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Biological role:

Industrial application:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility8.57 mg/mLNot Available
LogP-3.69HANSCH,C ET AL. (1995)
Predicted Properties
PropertyValueSource
Water Solubility80.6 g/LALOGPS
logP-3.5ALOGPS
logP-3.2ChemAxon
logS-0.26ALOGPS
pKa (Strongest Acidic)1.88ChemAxon
pKa (Strongest Basic)9.54ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area100.62 ŲChemAxon
Rotatable Bond Count4ChemAxon
Refractivity31.29 m³·mol⁻¹ChemAxon
Polarizability13.32 ųChemAxon
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) (3 TMS)splash10-0002-2950000000-2d6edc93ec5f8dee2223View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-002b-0940000000-4e285988bc537825d94dView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-00dj-9630000000-1ecc76aab86283892139View in MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-001i-8910000000-00f65ced0c55aa4ad169View in MoNA
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-0032-3980000000-3069de5b6c49e4176968View in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0002-0790000000-79d3e289c22cb183faa1View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-2950000000-2d6edc93ec5f8dee2223View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-002b-0940000000-4e285988bc537825d94dView in MoNA
GC-MSGC-MS Spectrum - GC-EI-QQ (Non-derivatized)splash10-00ea-6932100000-30d3f5dcc198a5971e96View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00dj-9630000000-1ecc76aab86283892139View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-001i-8910000000-00f65ced0c55aa4ad169View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0032-3980000000-3069de5b6c49e4176968View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0znc-9300000000-f88e86b78a4cee99a2d4View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00di-8290000000-f99e03763f636e557887View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-003r-6900000000-95b0a084dc076f9c7b91View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-001i-9000000000-c37d4c80a14ec029ef63View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0a59-9000000000-6f1888aa71bcb0adf76cView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-004j-0900000000-5fa8a338dcd2f2a6bdd2View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-004i-0900000000-16763200aa07f7629ad4View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-03di-3900000000-d9cfc5187aa799f6f978View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0a4i-0900000000-10ee9a593e13550bec1cView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0002-0900000000-4d045a3c1fc6e56f801bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-9000000000-c3c7f8f3754109a0c25bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-004i-0900000000-48bfae26c69408b7f0aeView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0002-0900000000-82c2a681e7522a7bb1d1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-0002-0905010000-af1c9ec4d0062fa6960eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-01q9-9700000000-2b967b896a6e48914512View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-004i-0900000000-1434321646181ea894a0View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-00di-0900000000-11fadb2530828eedad8aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-006t-0941100000-07d051890cb1d9e5c856View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-004i-9200000000-f8619d11f1f54d836bb1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-004i-0900000000-ea2dd00e79ef06e8dc04View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-00di-0900000000-3e239d4014c95a2ef873View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-0002-0900000000-b548959edce39d319cf4View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-0udi-0900000000-40d901f655797db2cd0fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-0udi-1900000000-f997527a39900ac431c7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-0udi-7900000000-60816f0601a4e6d25ffbView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0007-9000000000-6ee821f657c604d1afcaView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-000t-0900000000-7c02624abe56da9247a2View in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,1H] 2D NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Extracellular
  • Mitochondria
  • Lysosome
  • Endoplasmic reticulum
Biospecimen Locations
  • Blood
  • Cellular Cytoplasm
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Sweat
  • Urine
Tissue Location
  • Adipose Tissue
  • Adrenal Medulla
  • Epidermis
  • Fibroblasts
  • Intestine
  • Kidney
  • Muscle
  • Myelin
  • Nerve Cells
  • Neuron
  • Pancreas
  • Placenta
  • Platelet
  • Prostate
  • Skeletal Muscle
  • Spleen
  • Stratum Corneum
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified15-80 uMNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified10-80 uMInfant (1 - 3 months old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified8-80 uMChildren (3 months - 6 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified8-80 uMChildren (6 - 18 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified2-118 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified114.1 (75.2-203.1) uMInfant (0-1 year old)Not Specified
Normal
details
BloodDetected and Quantified90 +/- 65 uMInfant (0-1 year old)BothNormal details
BloodDetected and Quantified33.5 +/- 3.20 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified65.19 +/- 48.71 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified20.00-110.0 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified46.2 +/- 21.4 uMAdult (>18 years old)BothNormal details
BloodDetected but not Quantified Adult (>18 years old)Both
Normal
details
BloodDetected and Quantified24.0 (9.0-39.0) uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified176.9(140.6-218.8) uMChildren (1-13 uears old)Both
Normal
details
BloodDetected and Quantified145.0 +/- 6.0 uMAdult (>18 years old)MaleNormal details
BloodDetected and Quantified97.4 +/- 13.2 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified65.0 +/- 35.0 uMNewborn (0-30 days old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified35.0 +/- 14.0 uMChildren (1-13 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified37.0 +/- 14.0 uMChildren (1-13 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified60.0 +/- 16.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified46.0 +/- 13.0 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified33.0 +/- 12.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
Cellular CytoplasmDetected and Quantified1400 (1200-1600) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.143-0.298 uMChildren (1-13 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified40 +/- 52 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified1.8 +/- 0.7 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0 uMChildren (1-13 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified7.0 +/- 4.9 uMInfant (0-1 year old)Not Specified
Normal
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.34 +/- 0.14 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified32.6 +/- 6.9 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.44 +/- 0.29 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0-15 uMChildren (0 - 10 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0-15 uMAdolescent (>11 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.62 (0.18-1.15) uMAdult (>18 years old)BothNormal details
FecesDetected but not Quantified Infant (0-1 year old)Both
Normal
details
FecesDetected but not Quantified Children (1-13 years old)BothNormal details
FecesDetected but not Quantified Not SpecifiedBoth
Normal
details
FecesDetected but not Quantified Infant (0-1 year old)Both
Normal
details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedNormal details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)FemaleNormal details
FecesDetected and Quantified484.608 +/- 314.757 nmol/g wet fecesNot SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Not SpecifiedNormal details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)BothNormal details
FecesDetected but not Quantified Adult (>18 years old)BothNormal details
FecesDetected but not Quantified Adult (>18 years old)BothNormal details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)BothNormal details
SalivaDetected and Quantified13.6 +/- 2.4 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified13.3 +/- 6.0 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified12.6 +/- 6.5 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified11.6 +/- 6.9 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified>10 uMAdult (>18 years old)BothNormal details
SweatDetected and Quantified< 10 uMAdult (60 years old)Male
Normal
details
SweatDetected and Quantified< 10 uMAdult (40 years old)Male
Normal
details
SweatDetected but not Quantified Adult BothNormal details
UrineDetected and Quantified1.711 (0.329-3.092) umol/mmol creatinineAdult (>18 years old)BothNormal
    details
    UrineDetected and Quantified<4.76-217.61 umol/mmol creatinineAdult (>18 years old)BothNormal
      • David F. Putnam C...
    details
    UrineDetected and Quantified1.2-2.3 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
    UrineDetected and Quantified10.0 +/- 3.7 umol/mmol creatinineAdult (>18 years old)MaleNormal
      • Tanaka, T. and Na...
    details
    UrineDetected and Quantified8.5 (3.3-18.4) umol/mmol creatinineAdult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified0.5 (0.13-1.0) umol/mmol creatinineNewborn (0-30 days old)BothNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected but not Quantified Adult (>18 years old)Male
    Normal
    details
    UrineDetected but not Quantified Adult (>18 years old)Male
    Normal
    details
    UrineDetected and Quantified0 - 29.96 umol/mmol creatinineNewborn (0-30 days old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified2.04 - 29.06 umol/mmol creatinineInfant (1 - 6 months old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 17.98 umol/mmol creatinineInfant (6 months - <1 year old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 17.98 umol/mmol creatinineChildren (1 - 2 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 9.95 umol/mmol creatinineChildren (2 - 4 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 9.04 umol/mmol creatinineChildren (4 - 13 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 11.98 umol/mmol creatinineAdolescent (13 - 21 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 11.98 umol/mmol creatinineAdult (>21 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified6.4 (0.6-17.5) umol/mmol creatinineAdult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified1.1-1.7 umol/mmol creatinineAdult (>18 years old)MaleNormal details
    UrineDetected but not Quantified Adult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified13.3351 +/- 13.4213 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Normal
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified<11 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
    UrineDetected and Quantified1.38 umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected and Quantified30.83 +/- 23.237 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Normal
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified6.87 +/- 3.51 umol/mmol creatinineInfant (0-1 year old)BothNormal details
    Abnormal Concentrations
    BiospecimenStatusValueAgeSexConditionReferenceDetails
    BloodDetected and Quantified72 +/- 36.9 uMAdult (>18 years old)BothHeart Transplant details
    BloodDetected and Quantified32.7 +/- 18.0 uMAdult (>18 years old)MaleSchizophrenia details
    BloodDetected and Quantified34.7 +/- 26.0 uMAdult (>18 years old)BothSchizophrenia details
    BloodDetected and Quantified97.5 (60-241) uMChildren (1-13 years old)MaleLipoyltransferase 1 Deficiency details
    BloodDetected but not Quantified Adult (>18 years old)BothColorectal cancer details
    BloodDetected and Quantified48.4 (24.8-87.2) uMInfant (0-1 year old)MaleDicarboxylic Aminoaciduria details
    BloodDetected but not Quantified Adult (>18 years old)BothPancreatic Cancer details
    BloodDetected but not Quantified Adult (>18 years old)Bothoesophago-gastric cancer details
    BloodDetected but not Quantified Adult (>18 years old)Both
    gastric cancer
    details
    BloodDetected and Quantified109-296 uMNewborn (0-30 days old)MaleN-Acetylglutamate synthetase deficiency details
    BloodDetected and Quantified64-173 uMInfant (0-1 year old)Male
    N-Acetylglutamate synthetase deficiency
    details
    BloodDetected but not Quantified Adult (>18 years old)Both
    superficial gastric cancer
    details
    BloodDetected and Quantified71.3 +/- 12.3 uMAdult (>18 years old)Both
    Schizophrenia
    details
    BloodDetected and Quantified77.93 +/- 39.86 uMAdult (>18 years old)Both
    Schizophrenia
    details
    BloodDetected and Quantified251.7(156.9-334.7) uMChildren (1-13 uears old)Both
    Environmental enteric dysfunction
    details
    BloodDetected but not Quantified Adult (>18 years old)Both
    Schizophrenia
    details
    BloodDetected and Quantified29.8 (27.6-32.0) uMAdult (>18 years old)Both
    Epilepsy
    details
    BloodDetected and Quantified39.7 (36.5-42.9) uMChildren (1-13 years old)Both
    Epilepsy
    details
    BloodDetected and Quantified33.18 +/- 11.26 uMElderly (>65 years old)BothAlzheimer's disease details
    BloodDetected and Quantified56.6 (44.6-68.6) uMAdult (>18 years old)Both
    Epilepsy
    details
    BloodDetected and Quantified172.0 +/- 6.0 uMAdult (>18 years old)Both
    Heart failure
    details
    Cellular CytoplasmDetected and Quantified2500 (2100-2900) uMAdult (>18 years old)BothAnoxia details
    Cerebrospinal Fluid (CSF)Detected and Quantified0.213-0.539 uMChildren (1-13 years old)FemaleRett syndrome details
    Cerebrospinal Fluid (CSF)Detected and Quantified0.252-0.332 uMAdolescent (13-18 years old)FemaleRett syndrome details
    Cerebrospinal Fluid (CSF)Detected and Quantified0 uMChildren (1-13 years old)MaleLipoyltransferase 1 Deficiency details
    Cerebrospinal Fluid (CSF)Detected and Quantified7.5 uMInfant (0-1 year old)MaleDicarboxylic Aminoaciduria details
    Cerebrospinal Fluid (CSF)Detected and Quantified26.8 +/- 5.9 uMChildren (1-13 years old)Not SpecifiedLeukemia details
    Cerebrospinal Fluid (CSF)Detected and Quantified23.6 +/- 8.5 uMChildren (1-13 years old)Not Specified
    Leukemia
    details
    Cerebrospinal Fluid (CSF)Detected and Quantified0.32 +/- 0.09 uMAdult (>18 years old)BothSchizophrenia details
    FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
    FecesDetected but not Quantified Children (1-13 years old)BothAutism details
    FecesDetected but not Quantified Newborn (0-30 days old)Both
    Sepsis
    details
    FecesDetected but not Quantified Adult (>18 years old)Not Specifiedobese details
    FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedCrohns disease details
    FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedUlcerative colitis details
    FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedUnclassified IBD details
    FecesDetected but not Quantified Not SpecifiedNot Specified
    Cryptosporidium infection
    details
    FecesDetected but not Quantified Adult (>18 years old)FemaleMyalgic encephalomyelitis/chronic fatigue syndrome details
    FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Colorectal cancer
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Irritable bowel syndrome
    details
    FecesDetected but not Quantified Adult (>18 years old)BothColorectal cancer details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Crohn disease
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Ulcerative colitis
    details
    FecesDetected but not Quantified Adult (>18 years old)Not Specifiedasymptomatic diverticulosis details
    FecesDetected but not Quantified Adult (>18 years old)Not Specifiedsymptomatic uncomplicated diverticular disease details
    FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
    FecesDetected but not Quantified Adult (>18 years old)BothCrohns disease details
    FecesDetected but not Quantified Adult (>18 years old)BothUlcerative colitis details
    FecesDetected but not Quantified Adult (>18 years old)BothColorectal cancer details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Colorectal cancer
    details
    SalivaDetected but not Quantified Adult (>18 years old)BothOral cancer details
    SalivaDetected but not Quantified Adult (>18 years old)FemaleBreast cancer details
    SalivaDetected but not Quantified Adult (>18 years old)Not SpecifiedPancreatic cancer details
    SalivaDetected but not Quantified Adult (>18 years old)Not SpecifiedPeriodontal diseases details
    UrineDetected and Quantified0.0064 umol/mmol creatinineAdult (>18 years old)BothADPKD details
    UrineDetected but not Quantified Adult (>18 years old)BothColorectal cancer details
    UrineDetected but not Quantified Adult (>18 years old)Both
    Schizophrenia
    details
    UrineDetected but not Quantified Adult (>18 years old)Bothoesophago-gastric cancer details
    UrineDetected and Quantified11.2759 +/- 6.8729 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified20.6656 +/- 23.9161 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Gastroesophageal reflux disease
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified2 umol/mmol creatinineChildren (1-13 years old)MaleLipoyltransferase 1 Deficiency details
    UrineDetected and Quantified21.741 +/- 15.628 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    Associated Disorders and Diseases
    Disease References
    Alzheimer's disease
    1. Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed:17031479 ]
    Anoxia
    1. Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed:8579834 ]
    Autism
    1. De Angelis M, Piccolo M, Vannini L, Siragusa S, De Giacomo A, Serrazzanetti DI, Cristofori F, Guerzoni ME, Gobbetti M, Francavilla R: Fecal microbiota and metabolome of children with autism and pervasive developmental disorder not otherwise specified. PLoS One. 2013 Oct 9;8(10):e76993. doi: 10.1371/journal.pone.0076993. eCollection 2013. [PubMed:24130822 ]
    Autosomal dominant polycystic kidney disease
    1. Gronwald W, Klein MS, Zeltner R, Schulze BD, Reinhold SW, Deutschmann M, Immervoll AK, Boger CA, Banas B, Eckardt KU, Oefner PJ: Detection of autosomal dominant polycystic kidney disease by NMR spectroscopic fingerprinting of urine. Kidney Int. 2011 Jun;79(11):1244-53. doi: 10.1038/ki.2011.30. Epub 2011 Mar 9. [PubMed:21389975 ]
    Perillyl alcohol administration for cancer treatment
    1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
    Colorectal cancer
    1. Weir TL, Manter DK, Sheflin AM, Barnett BA, Heuberger AL, Ryan EP: Stool microbiome and metabolome differences between colorectal cancer patients and healthy adults. PLoS One. 2013 Aug 6;8(8):e70803. doi: 10.1371/journal.pone.0070803. Print 2013. [PubMed:23940645 ]
    2. Ni Y, Xie G, Jia W: Metabonomics of human colorectal cancer: new approaches for early diagnosis and biomarker discovery. J Proteome Res. 2014 Sep 5;13(9):3857-70. doi: 10.1021/pr500443c. Epub 2014 Aug 14. [PubMed:25105552 ]
    3. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ]
    4. Lin Y, Ma C, Liu C, Wang Z, Yang J, Liu X, Shen Z, Wu R: NMR-based fecal metabolomics fingerprinting as predictors of earlier diagnosis in patients with colorectal cancer. Oncotarget. 2016 May 17;7(20):29454-64. doi: 10.18632/oncotarget.8762. [PubMed:27107423 ]
    5. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
    6. Wang X, Wang J, Rao B, Deng L: Gut flora profiling and fecal metabolite composition of colorectal cancer patients and healthy individuals. Exp Ther Med. 2017 Jun;13(6):2848-2854. doi: 10.3892/etm.2017.4367. Epub 2017 Apr 20. [PubMed:28587349 ]
    Crohn's disease
    1. Bjerrum JT, Wang Y, Hao F, Coskun M, Ludwig C, Gunther U, Nielsen OH: Metabonomics of human fecal extracts characterize ulcerative colitis, Crohn's disease and healthy individuals. Metabolomics. 2015;11:122-133. Epub 2014 Jun 1. [PubMed:25598765 ]
    2. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
    3. Azario I, Pievani A, Del Priore F, Antolini L, Santi L, Corsi A, Cardinale L, Sawamoto K, Kubaski F, Gentner B, Bernardo ME, Valsecchi MG, Riminucci M, Tomatsu S, Aiuti A, Biondi A, Serafini M: Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I. Sci Rep. 2017 Aug 25;7(1):9473. doi: 10.1038/s41598-017-09958-9. [PubMed:28842642 ]
    Dicarboxylic aminoaciduria
    1. Melancon SB, Dallaire L, Lemieux B, Robitaille P, Potier M: Dicarboxylic aminoaciduria: an inborn error of amino acid conservation. J Pediatr. 1977 Sep;91(3):422-7. [PubMed:894411 ]
    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. [PubMed:14992292 ]
    Schizophrenia
    1. Alfredsson G, Wiesel FA: Monoamine metabolites and amino acids in serum from schizophrenic patients before and during sulpiride treatment. Psychopharmacology (Berl). 1989;99(3):322-7. [PubMed:2480613 ]
    2. 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. [PubMed:7595563 ]
    3. Fukushima T, Iizuka H, Yokota A, Suzuki T, Ohno C, Kono Y, Nishikiori M, Seki A, Ichiba H, Watanabe Y, Hongo S, Utsunomiya M, Nakatani M, Sadamoto K, Yoshio T: Quantitative analyses of schizophrenia-associated metabolites in serum: serum D-lactate levels are negatively correlated with gamma-glutamylcysteine in medicated schizophrenia patients. PLoS One. 2014 Jul 8;9(7):e101652. doi: 10.1371/journal.pone.0101652. eCollection 2014. [PubMed:25004141 ]
    4. Koike S, Bundo M, Iwamoto K, Suga M, Kuwabara H, Ohashi Y, Shinoda K, Takano Y, Iwashiro N, Satomura Y, Nagai T, Natsubori T, Tada M, Yamasue H, Kasai K: A snapshot of plasma metabolites in first-episode schizophrenia: a capillary electrophoresis time-of-flight mass spectrometry study. Transl Psychiatry. 2014 Apr 8;4:e379. doi: 10.1038/tp.2014.19. [PubMed:24713860 ]
    5. Yang J, Chen T, Sun L, Zhao Z, Qi X, Zhou K, Cao Y, Wang X, Qiu Y, Su M, Zhao A, Wang P, Yang P, Wu J, Feng G, He L, Jia W, Wan C: Potential metabolite markers of schizophrenia. Mol Psychiatry. 2013 Jan;18(1):67-78. doi: 10.1038/mp.2011.131. Epub 2011 Oct 25. [PubMed:22024767 ]
    Stomach cancer
    1. Yu L, Aa J, Xu J, Sun M, Qian S, Cheng L, Yang S, Shi R: Metabolomic phenotype of gastric cancer and precancerous stages based on gas chromatography time-of-flight mass spectrometry. J Gastroenterol Hepatol. 2011 Aug;26(8):1290-7. doi: 10.1111/j.1440-1746.2011.06724.x. [PubMed:21443661 ]
    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. [PubMed:16789974 ]
    Irritable bowel syndrome
    1. Ponnusamy K, Choi JN, Kim J, Lee SY, Lee CH: Microbial community and metabolomic comparison of irritable bowel syndrome faeces. J Med Microbiol. 2011 Jun;60(Pt 6):817-27. doi: 10.1099/jmm.0.028126-0. Epub 2011 Feb 17. [PubMed:21330412 ]
    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. [PubMed:15911239 ]
    Obesity
    1. Haro C, Montes-Borrego M, Rangel-Zuniga OA, Alcala-Diaz JF, Gomez-Delgado F, Perez-Martinez P, Delgado-Lista J, Quintana-Navarro GM, Tinahones FJ, Landa BB, Lopez-Miranda J, Camargo A, Perez-Jimenez F: Two Healthy Diets Modulate Gut Microbial Community Improving Insulin Sensitivity in a Human Obese Population. J Clin Endocrinol Metab. 2016 Jan;101(1):233-42. doi: 10.1210/jc.2015-3351. Epub 2015 Oct 27. [PubMed:26505825 ]
    Periodontal disease
    1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
    Rett syndrome
    1. Lappalainen R, Riikonen RS: High levels of cerebrospinal fluid glutamate in Rett syndrome. Pediatr Neurol. 1996 Oct;15(3):213-6. [PubMed:8916158 ]
    Sepsis
    1. Stewart CJ, Embleton ND, Marrs ECL, Smith DP, Fofanova T, Nelson A, Skeath T, Perry JD, Petrosino JF, Berrington JE, Cummings SP: Longitudinal development of the gut microbiome and metabolome in preterm neonates with late onset sepsis and healthy controls. Microbiome. 2017 Jul 12;5(1):75. doi: 10.1186/s40168-017-0295-1. [PubMed:28701177 ]
    Pancreatic cancer
    1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
    2. Xie G, Lu L, Qiu Y, Ni Q, Zhang W, Gao YT, Risch HA, Yu H, Jia W: Plasma metabolite biomarkers for the detection of pancreatic cancer. J Proteome Res. 2015 Feb 6;14(2):1195-202. doi: 10.1021/pr501135f. Epub 2014 Dec 8. [PubMed:25429707 ]
    N-acetylglutamate synthetase deficiency
    1. Guffon N, Vianey-Saban C, Bourgeois J, Rabier D, Colombo JP, Guibaud P: A new neonatal case of N-acetylglutamate synthase deficiency treated by carbamylglutamate. J Inherit Metab Dis. 1995;18(1):61-5. [PubMed:7623444 ]
    Diverticular disease
    1. Tursi A, Mastromarino P, Capobianco D, Elisei W, Miccheli A, Capuani G, Tomassini A, Campagna G, Picchio M, Giorgetti G, Fabiocchi F, Brandimarte G: Assessment of Fecal Microbiota and Fecal Metabolome in Symptomatic Uncomplicated Diverticular Disease of the Colon. J Clin Gastroenterol. 2016 Oct;50 Suppl 1:S9-S12. doi: 10.1097/MCG.0000000000000626. [PubMed:27622378 ]
    Eosinophilic esophagitis
    1. (). Mordechai, Hien, and David S. Wishart. .
    Lipoyltransferase 1 Deficiency
    1. Soreze Y, Boutron A, Habarou F, Barnerias C, Nonnenmacher L, Delpech H, Mamoune A, Chretien D, Hubert L, Bole-Feysot C, Nitschke P, Correia I, Sardet C, Boddaert N, Hamel Y, Delahodde A, Ottolenghi C, de Lonlay P: Mutations in human lipoyltransferase gene LIPT1 cause a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase. Orphanet J Rare Dis. 2013 Dec 17;8:192. doi: 10.1186/1750-1172-8-192. [PubMed:24341803 ]
    Ulcerative colitis
    1. Bjerrum JT, Wang Y, Hao F, Coskun M, Ludwig C, Gunther U, Nielsen OH: Metabonomics of human fecal extracts characterize ulcerative colitis, Crohn's disease and healthy individuals. Metabolomics. 2015;11:122-133. Epub 2014 Jun 1. [PubMed:25598765 ]
    2. Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
    3. Azario I, Pievani A, Del Priore F, Antolini L, Santi L, Corsi A, Cardinale L, Sawamoto K, Kubaski F, Gentner B, Bernardo ME, Valsecchi MG, Riminucci M, Tomatsu S, Aiuti A, Biondi A, Serafini M: Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I. Sci Rep. 2017 Aug 25;7(1):9473. doi: 10.1038/s41598-017-09958-9. [PubMed:28842642 ]
    Associated OMIM IDs
    DrugBank IDDB00142
    Phenol Explorer Compound IDNot Available
    FoodDB IDFDB012535
    KNApSAcK IDC00001358
    Chemspider ID30572
    KEGG Compound IDC00025
    BioCyc IDGLT
    BiGG ID33561
    Wikipedia LinkL-Glutamic_Acid
    METLIN ID5174
    PubChem Compound33032
    PDB IDGGL
    ChEBI ID16015
    References
    Synthesis Reference Horner, L.; Gross, A. Tertiary phosphines. IV. Use of phosphine imines in causing the introduction of primary amino groups. Liebigs Ann. Chem. (1955), 591 117-34.
    Material Safety Data Sheet (MSDS)Download (PDF)
    General References
    1. Molinari F, Raas-Rothschild A, Rio M, Fiermonte G, Encha-Razavi F, Palmieri L, Palmieri F, Ben-Neriah Z, Kadhom N, Vekemans M, Attie-Bitach T, Munnich A, Rustin P, Colleaux L: Impaired mitochondrial glutamate transport in autosomal recessive neonatal myoclonic epilepsy. Am J Hum Genet. 2005 Feb;76(2):334-9. Epub 2004 Dec 8. [PubMed:15592994 ]
    2. 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. doi: 10.1038/nature07762. [PubMed:19212411 ]
    3. Silwood CJ, Lynch E, Claxson AW, Grootveld MC: 1H and (13)C NMR spectroscopic analysis of human saliva. J Dent Res. 2002 Jun;81(6):422-7. [PubMed:12097436 ]
    4. Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed:8579834 ]
    5. Nicholson JK, O'Flynn MP, Sadler PJ, Macleod AF, Juul SM, Sonksen PH: Proton-nuclear-magnetic-resonance studies of serum, plasma and urine from fasting normal and diabetic subjects. Biochem J. 1984 Jan 15;217(2):365-75. [PubMed:6696735 ]
    6. 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. [PubMed:15911239 ]
    7. Cynober LA: Plasma amino acid levels with a note on membrane transport: characteristics, regulation, and metabolic significance. Nutrition. 2002 Sep;18(9):761-6. [PubMed:12297216 ]
    8. 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. [PubMed:14992292 ]
    9. Mross K, Maessen P, van der Vijgh WJ, Gall H, Boven E, Pinedo HM: Pharmacokinetics and metabolism of epidoxorubicin and doxorubicin in humans. J Clin Oncol. 1988 Mar;6(3):517-26. [PubMed:3162516 ]
    10. Noorlander CW, de Graan PN, Nikkels PG, Schrama LH, Visser GH: Distribution of glutamate transporters in the human placenta. Placenta. 2004 Jul;25(6):489-95. [PubMed:15135231 ]
    11. Grasselli G, Vigano L, Capri G, Locatelli A, Tarenzi E, Spreafico C, Bertuzzi A, Giani A, Materazzo C, Cresta S, Perotti A, Valagussa P, Gianni L: Clinical and pharmacologic study of the epirubicin and paclitaxel combination in women with metastatic breast cancer. J Clin Oncol. 2001 Apr 15;19(8):2222-31. [PubMed:11304775 ]
    12. Frayn KN, Khan K, Coppack SW, Elia M: Amino acid metabolism in human subcutaneous adipose tissue in vivo. Clin Sci (Lond). 1991 May;80(5):471-4. [PubMed:1851687 ]
    13. Alfredsson G, Wiesel FA, Tylec A: Relationships between glutamate and monoamine metabolites in cerebrospinal fluid and serum in healthy volunteers. Biol Psychiatry. 1988 Apr 1;23(7):689-97. [PubMed:2453224 ]
    14. Zoia C, Cogliati T, Tagliabue E, Cavaletti G, Sala G, Galimberti G, Rivolta I, Rossi V, Frattola L, Ferrarese C: Glutamate transporters in platelets: EAAT1 decrease in aging and in Alzheimer's disease. Neurobiol Aging. 2004 Feb;25(2):149-57. [PubMed:14749132 ]
    15. Rizzo V, Anesi A, Montalbetti L, Bellantoni G, Trotti R, Melzi d'Eril GV: Reference values of neuroactive amino acids in the cerebrospinal fluid by high-performance liquid chromatography with electrochemical and fluorescence detection. J Chromatogr A. 1996 Apr 5;729(1-2):181-8. [PubMed:9004939 ]
    16. Agnati LF, Ferre S, Lluis C, Franco R, Fuxe K: Molecular mechanisms and therapeutical implications of intramembrane receptor/receptor interactions among heptahelical receptors with examples from the striatopallidal GABA neurons. Pharmacol Rev. 2003 Sep;55(3):509-50. Epub 2003 Jul 17. [PubMed:12869660 ]
    17. Rutten EP, Engelen MP, Wouters EF, Schols AM, Deutz NE: Metabolic effects of glutamine and glutamate ingestion in healthy subjects and in persons with chronic obstructive pulmonary disease. Am J Clin Nutr. 2006 Jan;83(1):115-23. [PubMed:16400059 ]
    18. Agarwal A, Tripathi LM, Pandey VC: Status of ammonia, glutamate, lactate and pyruvate during Plasmodium yoelii infection and pyrimethamine treatment in mice. J Commun Dis. 1997 Sep;29(3):235-41. [PubMed:9465528 ]
    19. Heuschen UA, Allemeyer EH, Hinz U, Langer K, Heuschen G, Decker-Baumann C, Herfarth C, Stern J: Glutamine distribution in patients with ulcerative colitis and in patients with familial adenomatous polyposis coli before and after restorative proctocolectomy. Int J Colorectal Dis. 2002 Jul;17(4):245-52. Epub 2001 Dec 18. [PubMed:12073073 ]
    20. Olson RC: A proposed role for nerve growth factor in the etiology of multiple sclerosis. Med Hypotheses. 1998 Dec;51(6):493-8. [PubMed:10052870 ]
    21. Nakamura K, Matsumura K, Kobayashi S, Kaneko T: Sympathetic premotor neurons mediating thermoregulatory functions. Neurosci Res. 2005 Jan;51(1):1-8. [PubMed:15596234 ]
    22. Corrie JE, DeSantis A, Katayama Y, Khodakhah K, Messenger JB, Ogden DC, Trentham DR: Postsynaptic activation at the squid giant synapse by photolytic release of L-glutamate from a 'caged' L-glutamate. J Physiol. 1993 Jun;465:1-8. [PubMed:7901400 ]
    23. Reeds PJ, Burrin DG, Stoll B, Jahoor F: Intestinal glutamate metabolism. J Nutr. 2000 Apr;130(4S Suppl):978S-82S. [PubMed:10736365 ]
    24. Smith QR: Transport of glutamate and other amino acids at the blood-brain barrier. J Nutr. 2000 Apr;130(4S Suppl):1016S-22S. [PubMed:10736373 ]
    25. Okumoto S, Looger LL, Micheva KD, Reimer RJ, Smith SJ, Frommer WB: Detection of glutamate release from neurons by genetically encoded surface-displayed FRET nanosensors. Proc Natl Acad Sci U S A. 2005 Jun 14;102(24):8740-5. Epub 2005 Jun 6. [PubMed:15939876 ]
    26. Augustin H, Grosjean Y, Chen K, Sheng Q, Featherstone DE: Nonvesicular release of glutamate by glial xCT transporters suppresses glutamate receptor clustering in vivo. J Neurosci. 2007 Jan 3;27(1):111-23. [PubMed:17202478 ]

    Only showing the first 10 proteins. There are 94 proteins in total.

    Enzymes

    General function:
    Involved in oxidoreductase activity
    Specific function:
    Irreversible conversion of delta-1-pyrroline-5-carboxylate (P5C), derived either from proline or ornithine, to glutamate. This is a necessary step in the pathway interconnecting the urea and tricarboxylic acid cycles. The preferred substrate is glutamic gamma-semialdehyde, other substrates include succinic, glutaric and adipic semialdehydes.
    Gene Name:
    ALDH4A1
    Uniprot ID:
    P30038
    Molecular weight:
    55117.24
    Reactions
    1-Pyrroline-5-carboxylic acid + NAD(P)(+) + Water → L-Glutamic acid + NAD(P)Hdetails
    L-Glutamic gamma-semialdehyde + NAD + Water → L-Glutamic acid + NADH + Hydrogen Iondetails
    1-Pyrroline-5-carboxylic acid + NAD + Water → L-Glutamic acid + NADH + Hydrogen Iondetails
    1-Pyrroline-5-carboxylic acid + NADP + Water → L-Glutamic acid + NADPH + Hydrogen Iondetails
    General function:
    Involved in oxidoreductase activity
    Specific function:
    Maintains high levels of reduced glutathione in the cytosol.
    Gene Name:
    GSR
    Uniprot ID:
    P00390
    Molecular weight:
    56256.565
    General function:
    Involved in metabolic process
    Specific function:
    Controls the flux of glucose into the hexosamine pathway. Most likely involved in regulating the availability of precursors for N- and O-linked glycosylation of proteins.
    Gene Name:
    GFPT2
    Uniprot ID:
    O94808
    Molecular weight:
    76929.885
    Reactions
    L-Glutamine + Fructose 6-phosphate → L-Glutamic acid + Glucosamine 6-phosphatedetails
    General function:
    Involved in metabolic process
    Specific function:
    Controls the flux of glucose into the hexosamine pathway. Most likely involved in regulating the availability of precursors for N- and O-linked glycosylation of proteins.
    Gene Name:
    GFPT1
    Uniprot ID:
    Q06210
    Molecular weight:
    78805.81
    Reactions
    L-Glutamine + Fructose 6-phosphate → L-Glutamic acid + Glucosamine 6-phosphatedetails
    General function:
    Involved in 4-aminobutyrate transaminase activity
    Specific function:
    Catalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine.
    Gene Name:
    ABAT
    Uniprot ID:
    P80404
    Molecular weight:
    56438.405
    Reactions
    gamma-Aminobutyric acid + Oxoglutaric acid → Succinic acid semialdehyde + L-Glutamic aciddetails
    (S)-b-aminoisobutyric acid + Oxoglutaric acid → 2-Methyl-3-oxopropanoic acid + L-Glutamic aciddetails
    beta-Alanine + Oxoglutaric acid → Malonic semialdehyde + L-Glutamic aciddetails
    (S)-b-aminoisobutyric acid + Oxoglutaric acid → (S)-Methylmalonic acid semialdehyde + L-Glutamic aciddetails
    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. [PubMed: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. [PubMed:17016423 ]
    3. Imai H, Okuno T, Wu JY, Lee TJ: GABAergic innervation in cerebral blood vessels: an immunohistochemical demonstration of L-glutamic acid decarboxylase and GABA transaminase. J Cereb Blood Flow Metab. 1991 Jan;11(1):129-34. [PubMed:1983997 ]
    4. Happola O, Paivarinta H, Soinila S, Wu JY, Panula P: Localization of L-glutamate decarboxylase and GABA transaminase immunoreactivity in the sympathetic ganglia of the rat. Neuroscience. 1987 Apr;21(1):271-81. [PubMed:3299141 ]
    5. Far SR, Millimoria FR: Levels of glutamic acid decarboxylase (GAD), gamma amino butyric acid transaminase (GABA-T), glutamic acid dehydrogenase (GLDH) and proteins in cerebrospinal fluid of certain neurological disorders. Indian J Med Sci. 1996 Apr;50(4):99-102. [PubMed:8979623 ]
    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
    L-Tyrosine + Oxoglutaric acid → 4-Hydroxyphenylpyruvic acid + L-Glutamic aciddetails
    L-Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + L-Glutamic aciddetails
    2-Oxo-4-methylthiobutanoic acid + L-Glutamic acid → L-Methionine + Oxoglutaric aciddetails
    References
    1. Ozturk M, Chiu CY, Akdeniz N, Jenq SF, Chang SC, Hsa CY, Jap TS: Two novel mutations in the MEN1 gene in subjects with multiple endocrine neoplasia-1. J Endocrinol Invest. 2006 Jun;29(6):523-7. [PubMed:16840830 ]
    General function:
    Involved in transferase activity, transferring nitrogenous groups
    Specific function:
    Plays a key role in amino acid metabolism (By similarity).
    Gene Name:
    GOT1
    Uniprot ID:
    P17174
    Molecular weight:
    46247.14
    Reactions
    L-Aspartic acid + Oxoglutaric acid → Oxalacetic acid + L-Glutamic aciddetails
    L-Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + L-Glutamic aciddetails
    L-Tyrosine + Oxoglutaric acid → 4-Hydroxyphenylpyruvic acid + L-Glutamic aciddetails
    Cysteic acid + Oxoglutaric acid → 3-Sulfopyruvic acid + L-Glutamic aciddetails
    3-Sulfinoalanine + Oxoglutaric acid → B-Sulfinyl pyruvate + L-Glutamic aciddetails
    4-Hydroxy-L-glutamic acid + Oxoglutaric acid → D-4-Hydroxy-2-oxoglutarate + L-Glutamic aciddetails
    References
    1. Yudkoff M, Daikhin Y, Melo TM, Nissim I, Sonnewald U, Nissim I: The ketogenic diet and brain metabolism of amino acids: relationship to the anticonvulsant effect. Annu Rev Nutr. 2007;27:415-30. [PubMed:17444813 ]
    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). Plays a key role in amino acid metabolism. Important for metabolite exchange between mitochondria and cytosol. Facilitates cellular uptake of long-chain free fatty acids.
    Gene Name:
    GOT2
    Uniprot ID:
    P00505
    Molecular weight:
    47517.285
    Reactions
    L-Aspartic acid + Oxoglutaric acid → Oxalacetic acid + L-Glutamic aciddetails
    L-Kynurenine + Oxoglutaric acid → 4-(2-Aminophenyl)-2,4-dioxobutanoic acid + L-Glutamic aciddetails
    L-Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + L-Glutamic aciddetails
    L-Tyrosine + Oxoglutaric acid → 4-Hydroxyphenylpyruvic acid + L-Glutamic aciddetails
    Cysteic acid + Oxoglutaric acid → 3-Sulfopyruvic acid + L-Glutamic aciddetails
    3-Sulfinoalanine + Oxoglutaric acid → B-Sulfinyl pyruvate + L-Glutamic aciddetails
    4-Hydroxy-L-glutamic acid + Oxoglutaric acid → D-4-Hydroxy-2-oxoglutarate + L-Glutamic aciddetails
    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. [PubMed: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. [PubMed:17016423 ]
    3. Recasens M, Mandel P: Similarities between cysteinesulphinate transaminase and aspartate aminotransferase. Ciba Found Symp. 1979;(72):259-70. [PubMed:261660 ]
    4. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [PubMed:10592235 ]
    General function:
    Involved in catalytic activity
    Specific function:
    Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine.
    Gene Name:
    BCAT1
    Uniprot ID:
    P54687
    Molecular weight:
    38644.77
    Reactions
    L-Leucine + Oxoglutaric acid → Ketoleucine + L-Glutamic aciddetails
    L-Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + L-Glutamic aciddetails
    L-Valine + Oxoglutaric acid → alpha-Ketoisovaleric acid + L-Glutamic aciddetails
    References
    1. Smirnov SV, Samsonova NN, Novikova AE, Matrosov NG, Rushkevich NY, Kodera T, Ogawa J, Yamanaka H, Shimizu S: A novel strategy for enzymatic synthesis of 4-hydroxyisoleucine: identification of an enzyme possessing HMKP (4-hydroxy-3-methyl-2-keto-pentanoate) aldolase activity. FEMS Microbiol Lett. 2007 Aug;273(1):70-7. Epub 2007 Jun 6. [PubMed:17559390 ]
    General function:
    Involved in amidophosphoribosyltransferase activity
    Specific function:
    Not Available
    Gene Name:
    PPAT
    Uniprot ID:
    Q06203
    Molecular weight:
    57398.52
    Reactions
    5-Phosphoribosylamine + Pyrophosphate + L-Glutamic acid → L-Glutamine + Phosphoribosyl pyrophosphate + Waterdetails

    Transporters

    General function:
    Involved in transmembrane transport
    Specific function:
    Proton-linked monocarboxylate transporter. Catalyzes the rapid transport across the plasma membrane of many monocarboxylates such as lactate, pyruvate, branched-chain oxo acids derived from leucine, valine and isoleucine, and the ketone bodies acetoacetate, beta-hydroxybutyrate and acetate
    Gene Name:
    SLC16A1
    Uniprot ID:
    P53985
    Molecular weight:
    53957.7
    References
    1. Tamai I, Sai Y, Ono A, Kido Y, Yabuuchi H, Takanaga H, Satoh E, Ogihara T, Amano O, Izeki S, Tsuji A: Immunohistochemical and functional characterization of pH-dependent intestinal absorption of weak organic acids by the monocarboxylic acid transporter MCT1. J Pharm Pharmacol. 1999 Oct;51(10):1113-21. [PubMed:10579682 ]
    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. [PubMed:11278508 ]
    2. Kim DK, Kanai Y, Matsuo H, Kim JY, Chairoungdua A, Kobayashi Y, Enomoto A, Cha SH, Goya T, Endou H: The human T-type amino acid transporter-1: characterization, gene organization, and chromosomal location. Genomics. 2002 Jan;79(1):95-103. [PubMed:11827462 ]

    Only showing the first 10 proteins. There are 94 proteins in total.