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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-05-20 20:40:21 UTC
HMDB IDHMDB0000123
Secondary Accession Numbers
  • HMDB00123
Metabolite Identification
Common NameGlycine
DescriptionGlycine is a simple, nonessential amino acid, although experimental animals show reduced growth on low-glycine diets. The average adult ingests 3 to 5 grams of glycine daily. Glycine is involved in the body's production of DNA, phospholipids and collagen, and in release of energy. Glycine levels are effectively measured in plasma in both normal patients and those with inborn errors of glycine metabolism (http://www.dcnutrition.com/AminoAcids/). Nonketotic hyperglycinaemia (OMIM 606899 ) is an autosomal recessive condition caused by deficient enzyme activity of the glycine cleavage enzyme system (EC 2.1.1.10). The glycine cleavage enzyme system comprises four proteins: P-, T-, H- and L-proteins (EC 1.4.4.2, EC 2.1.2.10 and EC 1.8.1.4 for P-, T- and L-proteins). Mutations have been described in the GLDC (OMIM 238300 ), AMT (OMIM 238310 ), and GCSH (OMIM 238330 ) genes encoding the P-, T-, and H-proteins respectively. The glycine cleavage system catalyses the oxidative conversion of glycine into carbon dioxide and ammonia, with the remaining one-carbon unit transferred to folate as methylenetetrahydrofolate. It is the main catabolic pathway for glycine and it also contributes to one-carbon metabolism. Patients with a deficiency of this enzyme system have increased glycine in plasma, urine and cerebrospinal fluid (CSF) with an increased CSF: plasma glycine ratio (PMID 16151895 ). Glycine is also found to be associated with carbamoyl phosphate synthetase deficiency, iminoglycinuria, maple syrup urine disease, phenylketonuria, propionic acidemia, sarcosinemia, and tyrosinemia I, which are inborn errors of metabolism.
Structure
Thumb
Synonyms
ValueSource
Aminoacetic acidChEBI
AminoessigsaeureChEBI
Aminoethanoic acidChEBI
GChEBI
GlyChEBI
GlycinChEBI
GlycocollChEBI
GlykokollChEBI
GlyzinChEBI
H2N-CH2-COOHChEBI
HglyChEBI
LeimzuckerChEBI
AminoacetateGenerator
AminoethanoateGenerator
2-AminoacetateHMDB
2-Aminoacetic acidHMDB
AciportHMDB
amino-AcetateHMDB
amino-Acetic acidHMDB
GlicoaminHMDB
GlycolixirHMDB
GlycostheneHMDB
Gyn-hydralinHMDB
PadilHMDB
Glycine carbonate (1:1), monosodium saltMeSH
Glycine carbonate (2:1), monopotassium saltMeSH
Glycine sulfate (3:1)MeSH
Glycine, monoammonium saltMeSH
Glycine, monosodium saltMeSH
Glycine, sodium hydrogen carbonateMeSH
Monoammonium salt glycineMeSH
Calcium salt glycineMeSH
Glycine hydrochloride (2:1)MeSH
Glycine phosphate (1:1)MeSH
Glycine, monopotasssium saltMeSH
Monopotasssium salt glycineMeSH
Monosodium salt glycineMeSH
Glycine carbonate (2:1), monolithium saltMeSH
Glycine carbonate (2:1), monosodium saltMeSH
Glycine hydrochlorideMeSH
Glycine, copper saltMeSH
Hydrochloride, glycineMeSH
Salt glycine, monoammoniumMeSH
Acid, aminoaceticMeSH
Cobalt salt glycineMeSH
Copper salt glycineMeSH
Glycine phosphateMeSH
Glycine, calcium saltMeSH
Glycine, calcium salt (2:1)MeSH
Glycine, cobalt saltMeSH
Phosphate, glycineMeSH
Salt glycine, monosodiumMeSH
Chemical FormulaC2H5NO2
Average Molecular Weight75.0666
Monoisotopic Molecular Weight75.032028409
IUPAC Name2-aminoacetic acid
Traditional Nameglycine
CAS Registry Number56-40-6
SMILES
NCC(O)=O
InChI Identifier
InChI=1S/C2H5NO2/c3-1-2(4)5/h1,3H2,(H,4,5)
InChI KeyDHMQDGOQFOQNFH-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as alpha amino acids. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon).
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentAlpha amino acids
Alternative Parents
Substituents
  • Alpha-amino acid
  • Amino acid
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Organic nitrogen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Organopnictogen compound
  • Primary aliphatic amine
  • Organic oxygen compound
  • Carbonyl group
  • Amine
  • 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 Point262.2 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility249 mg/mLNot Available
LogP-3.21HANSCH,C ET AL. (1995)
Predicted Properties
PropertyValueSource
Water Solubility552 g/LALOGPS
logP-3.3ALOGPS
logP-3.4ChemAxon
logS0.87ALOGPS
pKa (Strongest Acidic)2.31ChemAxon
pKa (Strongest Basic)9.24ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area63.32 ŲChemAxon
Rotatable Bond Count1ChemAxon
Refractivity16 m³·mol⁻¹ChemAxon
Polarizability6.65 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)splash10-00dj-2900000000-0ef96bcf06ce475afcddView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-00dj-1900000000-1d289099ac79cfb8bb19View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-00di-7910000000-6c972a683dfb75b69331View in MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-0udi-0900000000-ef69e38ee6cebc2ece00View in MoNA
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-00di-2910000000-3215b9e40f20c7b306cdView in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-001i-9000000000-719b7f248956f13a312dView in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0udi-0900000000-99b4fc43740b21edc786View in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-00di-1910000000-4cff4d14c73acff9442fView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00dj-2900000000-0ef96bcf06ce475afcddView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00dj-1900000000-1d289099ac79cfb8bb19View in MoNA
GC-MSGC-MS Spectrum - GC-EI-QQ (Non-derivatized)splash10-0002-4960000000-2c6fa028e985c6019854View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00di-7910000000-6c972a683dfb75b69331View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00di-2910000000-3215b9e40f20c7b306cdView in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0udi-0900000000-ef69e38ee6cebc2ece00View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00ds-2900000000-ffffed9c78c16a884e4aView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-004r-3900000000-f288b50b7b6890429811View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0udi-1900000000-c76140c31c1f120e4b9dView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-001i-9000000000-f0a2cfbefb9fcd9b6c3eView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00di-9300000000-b8bbfc1276d5adb1ba04View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-00di-9000000000-6001578fc511ba3fefefView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-00di-9000000000-79b2a0a9d93de6a62358View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-00di-9000000000-9290dbe208c4744f4431View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00di-9000000000-6001578fc511ba3fefefView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00di-9000000000-79b2a0a9d93de6a62358View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00di-9000000000-9290dbe208c4744f4431View in MoNA
LC-MS/MSLC-MS/MS Spectrum - , negativesplash10-00di-9000000000-605b44ac311a9af4bb7aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-003r-9000000000-725357e461c898a7451eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-001i-9000000000-9f3930e66b117ad91dcaView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-001i-9000000000-b3336097dddbb5e22871View in MoNA
LC-MS/MSLC-MS/MS Spectrum - EI-B (HITACHI RMU-6M) , Positivesplash10-001i-9000000000-719b7f248956f13a312dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-004i-9000000000-342ab462db0835abb3d2View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-0ar1-9010000000-9daadc1d169a8530926dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-07y0-9220000000-8c7785f1f3aa8052679fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-0ula-9110000000-43ada06fe1b56b4e9fccView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-017i-9000000000-fbd78fbb48f082235f42View in MoNA
LC-MS/MSLC-MS/MS Spectrum - CE-ESI-TOF (CE-system connected to 6210 Time-of-Flight MS, Agilent) , Positivesplash10-004i-9000000000-c38d0fb28793438083a9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - DI-ESI-Q-Exactive Plus , Positivesplash10-004i-9000000000-18a7ae48c7b0e15cdf18View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-004i-9000000000-342ab462db0835abb3d2View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-0ar1-9010000000-9daadc1d169a8530926dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-07y0-9220000000-8c7785f1f3aa8052679fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-0ula-9110000000-43ada06fe1b56b4e9fccView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-017i-9000000000-fbd78fbb48f082235f42View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-00di-9000000000-89b2c043a5afe3ebc6f6View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-00di-9000000000-b4046e208ee8adb87021View in MoNA
MSMass Spectrum (Electron Ionization)splash10-001i-9000000000-222d6c3a1ba6afcd7ea9View in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H 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
  • Peroxisome
Biospecimen Locations
  • Bile
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Sweat
  • Urine
Tissue Locations
  • Bladder
  • Brain
  • Epidermis
  • Fibroblasts
  • Intestine
  • Kidney
  • Myelin
  • Neuron
  • Pancreas
  • Placenta
  • Platelet
  • Prostate
  • Spleen
  • Stratum Corneum
  • Thyroid Gland
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BileDetected and Quantified>10 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified242.0 +/- 44.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified258.0 +/- 64.0 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified106-272 uMNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified150-350 uMInfant (1 - 3 months old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified125-400 uMChildren (3 months - 6 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified140-490 uMChildren (6 - 18 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified329.9 +/- 105.6 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified147-321 uMNot SpecifiedNot SpecifiedNormal details
BloodDetected but not Quantified Adult (>18 years old)Both
Normal
details
BloodDetected and Quantified149-301 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified184-356 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified140-420 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified200-600 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified80-341 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified100-320 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified126-384 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified280.0 (140.0-420.0) uMNewborn (0-30 days old)BothNormal details
BloodDetected and Quantified<300 uMChildren (1 - 13 years old)MaleNormal details
BloodDetected and Quantified100 - 310 uMChildren (1 - 13 years old)Not SpecifiedNormal details
BloodDetected and Quantified120-320 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified213 +/- 35 uMInfant (0-1 year old)BothNormal details
BloodDetected and Quantified56-308 uMInfant (0-1 year old)BothNormal details
BloodDetected and Quantified60-310 uMChildren (1-13 years old)BothNormal details
BloodDetected and Quantified180-709 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified212.4 +/- 57.4 uMAdult (>18 years old)MaleNormal details
BloodDetected and Quantified150.0-440.0 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified220 +/- 33 uMChildren (1 - 13 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified300 +/- 114 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified255.4 +/- 65.9 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified6793.967 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified236 +/- 43 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified304.4(255.1-362.8) uMChildren (1-13 uears old)Both
Normal
details
BloodDetected and Quantified230.0 (178.0-282.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified325.4 +/- 126.8 uMAdult (>18 years old)BothNormal details
BloodDetected but not Quantified Adult (>18 years old)Both
Normal
details
BloodDetected and Quantified460.0 +/- 275.0 uMNewborn (0-30 days old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified234.0 +/- 34.0 uMChildren (1-13 years old)MaleNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified7011 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified13-Feb uMNot SpecifiedNot SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified<10 uMNewborn (0-30 days old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified1-16 uMChildren (1-13 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified7-15 uMNot SpecifiedNot SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified3-10 uMInfant (0-1 year old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified3-10 uMNewborn (0-30 days old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified3.6-9 uMInfant (0-1 year old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified3-7 uMChildren (1-13 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified4.7 +/- 1.5 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified4-14 uMNewborn (0-30 days old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified23.0-39.5 uMChildren (1-13 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified5.9-8.5 uMNewborn (0-30 days old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified9.0 (3.0-19.0) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified9.5 +/- 4.7 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified8.2 +/- 3.0 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified6.1 +/- 1.4 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified4.63 +/- 0.74 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified1-10 uMChildren (0 - 10 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified1-10 uMAdolescent (>11 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified8.30 (5.88-10.7) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified11 +/- 4  uMNot SpecifiedNot SpecifiedNormal 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 Adult (>18 years old)BothNormal details
FecesDetected but not Quantified Not SpecifiedBoth
Normal
details
FecesDetected but not Quantified Children (6 - 18 years old)BothNormal details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedNormal details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
FecesDetected but not Quantified Adult (>18 years old)FemaleNormal 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 Specified
Normal
details
FecesDetected and Quantified400 +/- 290 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified150 +/- 170 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)FemaleNormal 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)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)BothNormal details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Not 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)MaleNormal details
SalivaDetected and Quantified17 (1-122) uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified27 (5-447) uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified28 (5-130) uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified17 (3-132) uMAdult (>18 years old)Female
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)BothNormal details
SalivaDetected and Quantified92.0 +/- 83.7 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified18.6 +/- 16.4 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified1-472 uMAdult (>18 years old)Male
normal
details
SalivaDetected and Quantified1-94 uMAdult (>18 years old)Male
normal
details
SalivaDetected and Quantified39.2 +/- 9.3 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified31.0 +/- 14.7 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified23.2 +/- 11.6 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified177.80 +/- 143.20 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected and Quantified27.7 +/- 25.9 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified41.0 +/- 18.0 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified46.7 +/- 18.1 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified187.75 +/- 120.02 uMAdult (>18 years old)FemaleNormal details
SalivaDetected and Quantified>10 uMAdult (>18 years old)BothNormal details
SweatDetected and Quantified< 10 uMAdult (60 years old)Male
Normal
details
SweatDetected and Quantified320 uMAdult (40 years old)Male
Normal
details
SweatDetected but not Quantified Adult BothNormal details
UrineDetected and Quantified75.7 +/- 51.7 umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified165.0 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified151(48-233) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected but not Quantified Adult (>18 years old)Both
Normal
details
UrineDetected and Quantified119.95-599.74 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
details
UrineDetected and Quantified151.0 (233-248) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified35-95 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified513.54 +/- 360.61 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified<300 umol/mmol creatinineChildren (1 - 13 years old)MaleNormal details
UrineDetected and Quantified<525 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0-198 umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0-379 umol/mmol creatinineInfant (0-1 year old)Both
Normal
details
UrineDetected and Quantified254.24-2341.24 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified43-173 umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified60-190 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified64-165 umol/mmol creatinineInfant (0-1 year old)Both
Normal
details
UrineDetected and Quantified106 (44-300) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified71.23 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Shaykhutdinov RA,...
details
UrineDetected and Quantified128.1 +/- 65.5 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified187.6 +/- 92.3 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified459.36 - 1767.47 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified210.07 - 1198.66 umol/mmol creatinineInfant (1 - 6 months old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified187.91 - 719.74 umol/mmol creatinineInfant (6 months - <1 year old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified181.35 - 576.72 umol/mmol creatinineChildren (1 - 2 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified182.93 - 528.33 umol/mmol creatinineChildren (2 - 4 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified107.41 - 400.01 umol/mmol creatinineChildren (4 - 13 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified15.0 (10.0-23.0) umol/mmol creatinineNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified73.83 - 282.65 umol/mmol creatinineAdolescent (13 - 21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified30.0 +/- 19.0 umol/mmol creatinineChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified73.83 - 282.65 umol/mmol creatinineAdult (>21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified92.0 +/- 39.0 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified139.0 +/- 86.0 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified136.48 +/- 77.27 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified101 (37.0-250.6) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified527.145 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified34-83 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified110-356 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified118.9 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified345.9154 +/- 161.9379 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified312.536 +/- 214.068 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified229.8 +/- 50.7 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
BloodDetected and Quantified238.0 +/- 45.5 uMChildren (1-13 years old)Both
Obesity
    • Metabolomics reve...
details
BloodDetected and Quantified234.9 +/- 181.1 uMAdult (>18 years old)BothHeart Transplant details
BloodDetected and Quantified175-252 uMAdolescent (13-18 years old)Both
3-Phosphoglycerate Dehydrogenase Deficiency
details
BloodDetected and Quantified263.5 (114-356) uMChildren (1-13 years old)MaleLipoyltransferase 1 Deficiency details
BloodDetected and Quantified63 uMNewborn (0-30 days old)MaleNeu-Laxova Syndrome 1 details
BloodDetected and Quantified261.6 (99.4) uMAdult (>18 years old)Female
Pregnancy with fetuses with trisomy 18
details
BloodDetected and Quantified209.8 (74.9) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified121 uMInfant (0-1 year old)MalePhosphoserine Aminotransferase Deficiency details
BloodDetected and Quantified110 uMNewborn (0-30 days old)FemalePhosphoserine Aminotransferase Deficiency details
BloodDetected and Quantified188.0 (42.8) uMAdult (>18 years old)FemaleEarly preeclampsia details
BloodDetected and Quantified77-97 uMInfant (0-1 year old)MalePhosphoserine Phosphatase Deficiency details
BloodDetected and Quantified239.6 (106.1) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified906 uMNewborn (0-30 days old)MaleHyperglycinemia, lactic acidosis, and seizures details
BloodDetected and Quantified228-742 uMNewborn (0-30 days old)BothPyridoxamine 5-prime-phosphate oxidase deficiency details
BloodDetected and Quantified1090.0 (780.0-1400.0) uMNewborn (0-30 days old)BothNon-ketotic hyperglycinemia details
BloodDetected but not Quantified Adult (>18 years old)BothColorectal cancer details
BloodDetected and Quantified486.0 (232.0-740.0) uMAdult (>18 years old)BothD-Glyceric acidemia
    • MetaGene: Metabol...
details
BloodDetected and Quantified1000.0 (800.0-1200.0) uMChildren (1-13 years old)BothD-Glyceric acidura
    • MetaGene: Metabol...
details
BloodDetected and Quantified290 uMChildren (1 - 13 years old)Female3-Methylcrotonyl-CoA Carboxylase Deficiency details
BloodDetected and Quantified354.6 uMInfant (0-1 year old)Female
Sarcosinemia
details
BloodDetected and Quantified122-258 uMInfant (0-1 year old)BothIminoglycinuria details
BloodDetected and Quantified126-378 uMChildren (1-13 years old)Male
N-acetylglutamate synthetase deficiency
details
BloodDetected and Quantified214 +/- 76 uMChildren (1 - 13 years old)MaleHistidinuria details
BloodDetected and Quantified254-310 uMAdult (>18 years old)Male
Sarcosinemia
details
BloodDetected and Quantified880-1070 uMChildren (1 - 13 years old)MaleHyperglycinaemia details
BloodDetected and Quantified105 uMNewborn (0-30 days old)MaleNeu-Laxova Syndrome 1 details
BloodDetected and Quantified141.82 (37.97) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified135.06 (40.06) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified238.4 (129.3) uMAdult (>18 years old)FemaleLate-onset preeclampsia details
BloodDetected and Quantified244.0 (115.7) uMAdult (>18 years old)FemalePregnancy details
BloodDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
BloodDetected and Quantified327.8(265.0-397.5) uMChildren (1-13 uears old)Both
Environmental enteric dysfunction
details
BloodDetected and Quantified3463.591 uMChildren (1-13 years old)MaleGlucoglycinuria details
BloodDetected and Quantified210.0 (203.0-217.0) uMAdult (>18 years old)BothRefractory localization-related epilepsy (RLE) details
BloodDetected and Quantified354.0 (309.0-399.0) uMChildren (1-13 years old)BothJuvenile myoclonic epilepsy (JME) details
BloodDetected and Quantified0.153 +/- 0.012 uMElderly (>65 years old)BothAlzheimer's disease details
BloodDetected and Quantified5.1 (2.5) uMAdult (>18 years old)FemaleDown syndrome pregnancy details
BloodDetected and Quantified4.1 (1.7) uMAdult (>18 years old)FemalePregnancy details
BloodDetected but not Quantified Adult (>18 years old)Both
Schizophrenia
details
Cerebrospinal Fluid (CSF)Detected and Quantified6-7 uMAdult (>18 years old)Male
Sarcosinemia
details
Cerebrospinal Fluid (CSF)Detected and Quantified5 uMAdolescent (13-18 years old)Male
3-Phosphoglycerate Dehydrogenase Deficiency
details
Cerebrospinal Fluid (CSF)Detected and Quantified368(83-1927) uMNewborn (0-30 days old)Not SpecifiedNonketotic Hyperglycinemia details
Cerebrospinal Fluid (CSF)Detected and Quantified300-1500 uMChildren (1-13 years old)MaleD-Glyceric acidura details
Cerebrospinal Fluid (CSF)Detected and Quantified5 uMChildren (1-13 years old)MaleLipoyltransferase 1 Deficiency details
Cerebrospinal Fluid (CSF)Detected and Quantified6 uMNewborn (0-30 days old)MaleNeu-Laxova Syndrome 1 details
Cerebrospinal Fluid (CSF)Detected and Quantified<1 uMInfant (0-1 year old)MalePhosphoserine Aminotransferase Deficiency details
Cerebrospinal Fluid (CSF)Detected and Quantified<1 uMNewborn (0-30 days old)FemalePhosphoserine Aminotransferase Deficiency details
Cerebrospinal Fluid (CSF)Detected and Quantified48.6 uMChildren (1-13 years old)FemaleEpilepsy, early-onset, vitamin B6-dependent details
Cerebrospinal Fluid (CSF)Detected and Quantified2.8 uMInfant (0-1 year old)MalePhosphoserine Phosphatase Deficiency details
Cerebrospinal Fluid (CSF)Detected and Quantified11 uMNewborn (0-30 days old)FemaleEpilepsy, early-onset, vitamin B6-dependent details
Cerebrospinal Fluid (CSF)Detected and Quantified3 uMChildren (1-13 years old)MalePhosphoserine Phosphatase Deficiency details
Cerebrospinal Fluid (CSF)Detected and Quantified16-66 uMNewborn (0-30 days old)BothPyridoxamine 5-prime-phosphate oxidase deficiency details
Cerebrospinal Fluid (CSF)Detected and Quantified9.7 +/- 4.9 uMChildren (1-13 years old)Not SpecifiedLeukemia details
Cerebrospinal Fluid (CSF)Detected and Quantified13.4 +/- 6.4 uMChildren (1-13 years old)Not Specified
Leukemia
details
Cerebrospinal Fluid (CSF)Detected and Quantified9.0405 (8.716-9.623) uMNot SpecifiedNot Specifiedcontrol details
Cerebrospinal Fluid (CSF)Detected and Quantified4.5 +/- 0.75 uMAdult (>18 years old)BothSchizophrenia details
Cerebrospinal Fluid (CSF)Detected and Quantified0.28 +/- 0.12 uMAdult (>18 years old)BothAlzheimer's disease details
FecesDetected but not Quantified Children (1-13 years old)BothAutism details
FecesDetected but not Quantified Children (6 - 18 years old)BothCrohns disease details
FecesDetected but not Quantified Children (6 - 18 years old)BothUlcerative colitis 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 Adult (>18 years old)Both
Irritable bowel syndrome
details
FecesDetected but not Quantified Adult (>18 years old)Both
Ulcerative colitis
details
FecesDetected but not Quantified Adult (>18 years old)FemaleMyalgic encephalomyelitis/chronic fatigue syndrome details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not Quantified Adult (>18 years old)BothColorectal cancer 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)Both
Ulcerative colitis
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
FecesDetected but not Quantified Adult (>18 years old)MaleGout details
FecesDetected but not Quantified Adult (>18 years old)BothColorectal 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
SalivaDetected and Quantified164.24 +/- 54.66 uMAdult (>18 years old)MaleAlzheimer's disease details
SalivaDetected and Quantified120.76 +/- 85.97 uMAdult (>18 years old)MaleFrontotemporal lobe dementia details
SalivaDetected and Quantified147.29 +/- 61.58 uMAdult (>18 years old)BothLewy body disease details
UrineDetected and Quantified50.266-146.448 umol/mmol creatinineInfant (0-1 year old)FemaleCarbamoyl Phosphate Synthetase Deficiency details
UrineDetected but not Quantified Adult (>18 years old)Both
Schizophrenia
details
UrineDetected and Quantified3000.0 (1000.0-5000.0) umol/mmol creatinineChildren (1-13 years old)BothCarbamoyl phosphate synthetase deficiency
    • MetaGene: Metabol...
details
UrineDetected and Quantified56 umol/mmol creatinineAdult (>18 years old)Not Specified
Propionic acidemia
details
UrineDetected and Quantified972.85 umol/mmol creatinineInfant (0-1 year old)Female
Sarcosinemia
details
UrineDetected and Quantified104-494 umol/mmol creatinineAdult (>18 years old)BothHyperglycinuria details
UrineDetected and Quantified160-632 umol/mmol creatinineAdolescent (13-18 years old)BothHyperglycinuria details
UrineDetected and Quantified1609-1747 umol/mmol creatinineAdult (>18 years old)Female
Iminoglycinuria
details
UrineDetected and Quantified198-419 umol/mmol creatinineAdult (>18 years old)Male
Sarcosinemia
details
UrineDetected and Quantified4753-6114 umol/mmol creatinineChildren (1 - 13 years old)MaleHyperglycinaemia details
UrineDetected and Quantified576-1965 umol/mmol creatinineChildren (1-13 years old)BothIminoglycinuria details
UrineDetected and Quantified0.0024 - 0.0339 umol/mmol creatinineAdult (>18 years old)BothADPKD details
UrineDetected and Quantified1039.229 umol/mmol creatinineChildren (1-13 years old)MaleGlucoglycinuria details
UrineDetected and Quantified84.0 +/- 67.0 umol/mmol creatinineAdult (>18 years old)BothLung cancer details
UrineDetected and Quantified0.010 +/- 0.001 umol/mmol creatinineAdult (>18 years old)BothAlzheimer's disease details
UrineDetected and Quantified229 umol/mmol creatinineChildren (1-13 years old)MaleLipoyltransferase 1 Deficiency details
UrineDetected and Quantified178.2 +/- 6.4 umol/mmol creatinineAdult (>18 years old)Both3-Hydroxy-3- methylglutaryl-CoA lyase (HL) deficency details
UrineDetected and Quantified135.8 +/- 5.8 umol/mmol creatinineAdult (>18 years old)Both3-Hydroxy-3- methylglutaryl-CoA lyase (HL) deficency details
UrineDetected and Quantified53.1 +/- 1.8 umol/mmol creatinineAdult (>18 years old)BothArgininosuccinic aciduria (ASL) details
UrineDetected and Quantified585.5 +/- 32.1 umol/mmol creatinineAdult (>18 years old)BothPropionic acidemia details
UrineDetected and Quantified550.00 (100.00-1000.00) umol/mmol creatinineChildren (1-13 years old)BothIminoglycinuria
    • MetaGene: Metabol...
details
UrineDetected and Quantified2040.3 +/- 202.6 umol/mmol creatinineAdult (>18 years old)BothPropionic acidemia details
UrineDetected and Quantified49.9 +/- 1.3 umol/mmol creatinineAdult (>18 years old)BothTyrosinemia I details
UrineDetected and Quantified295.0484 +/- 155.5952 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified176.2 +/- 9.2 umol/mmol creatinineAdult (>18 years old)BothTyrosinemia I details
UrineDetected and Quantified253.9195 +/- 254.6574 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Mordechai, Hien, ...
details
UrineDetected and Quantified82.2 +/- 4.1 umol/mmol creatinineAdult (>18 years old)BothPhenylketonuria details
UrineDetected and Quantified304.8 +/- 11.3 umol/mmol creatinineAdult (>18 years old)BothPhenylketonuria details
UrineDetected and Quantified233.7 +/- 10.5 umol/mmol creatinineAdult (>18 years old)BothMaple syrup urine disease details
UrineDetected and Quantified3205.1 +/- 83.4 umol/mmol creatinineAdult (>18 years old)BothAminoaciduria details
UrineDetected and Quantified232.913 +/- 149.591 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
Associated Disorders and Diseases
Disease References
3-Phosphoglycerate dehydrogenase deficiency
  1. Tabatabaie L, Klomp LW, Rubio-Gozalbo ME, Spaapen LJ, Haagen AA, Dorland L, de Koning TJ: Expanding the clinical spectrum of 3-phosphoglycerate dehydrogenase deficiency. J Inherit Metab Dis. 2011 Feb;34(1):181-4. doi: 10.1007/s10545-010-9249-5. Epub 2010 Nov 27. [PubMed:21113737 ]
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 ]
  2. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Pregnancy
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
  2. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ]
  3. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester Down syndrome prediction. Am J Obstet Gynecol. 2013 May;208(5):371.e1-8. doi: 10.1016/j.ajog.2012.12.035. Epub 2013 Jan 8. [PubMed:23313728 ]
  4. Bahado-Singh RO, Akolekar R, Chelliah A, Mandal R, Dong E, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester trisomy 18 detection. Am J Obstet Gynecol. 2013 Jul;209(1):65.e1-9. doi: 10.1016/j.ajog.2013.03.028. Epub 2013 Mar 25. [PubMed:23535240 ]
  5. Bahado-Singh RO, Ertl R, Mandal R, Bjorndahl TC, Syngelaki A, Han B, Dong E, Liu PB, Alpay-Savasan Z, Wishart DS, Nicolaides KH: Metabolomic prediction of fetal congenital heart defect in the first trimester. Am J Obstet Gynecol. 2014 Sep;211(3):240.e1-240.e14. doi: 10.1016/j.ajog.2014.03.056. Epub 2014 Apr 1. [PubMed:24704061 ]
Argininosuccinic aciduria
  1. Gronwald W, Klein MS, Kaspar H, Fagerer SR, Nurnberger N, Dettmer K, Bertsch T, Oefner PJ: Urinary metabolite quantification employing 2D NMR spectroscopy. Anal Chem. 2008 Dec 1;80(23):9288-97. doi: 10.1021/ac801627c. [PubMed:19551947 ]
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 ]
3-Methyl-crotonyl-glycinuria
  1. Rutledge SL, Berry GT, Stanley CA, van Hove JL, Millington D: Glycine and L-carnitine therapy in 3-methylcrotonyl-CoA carboxylase deficiency. J Inherit Metab Dis. 1995;18(3):299-305. [PubMed:7474896 ]
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 ]
Carbamoyl Phosphate Synthetase Deficiency
  1. Freeman JM, Nicholson JF, Schimke RT, Rowland LP, Carter S: Congenital hyperammonemia. Association with hyperglycinemia and decreased levels of carbamyl phosphate synthetase. Arch Neurol. 1970 Nov;23(5):430-7. [PubMed:5471650 ]
  2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
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. Ritchie SA, Ahiahonu PW, Jayasinghe D, Heath D, Liu J, Lu Y, Jin W, Kavianpour A, Yamazaki Y, Khan AM, Hossain M, Su-Myat KK, Wood PL, Krenitsky K, Takemasa I, Miyake M, Sekimoto M, Monden M, Matsubara H, Nomura F, Goodenowe DB: Reduced levels of hydroxylated, polyunsaturated ultra long-chain fatty acids in the serum of colorectal cancer patients: implications for early screening and detection. BMC Med. 2010 Feb 15;8:13. doi: 10.1186/1741-7015-8-13. [PubMed:20156336 ]
  3. 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 ]
  4. 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 ]
  5. 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 ]
  6. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
  7. 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 ]
D-Glyceric acidemia
  1. Boneh A, Degani Y, Harari M: Prognostic clues and outcome of early treatment of nonketotic hyperglycinemia. Pediatr Neurol. 1996 Sep;15(2):137-41. [PubMed:8888048 ]
  2. Brandt NJ, Brandt S, Rasmussen K, Schnoheyder F: Letter: Hyperglycericacidaemia with hyperglycinaemia: a new inborn error of metabolism. Br Med J. 1974 Nov 9;4(5940):344. [PubMed:4434100 ]
  3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
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. [PubMed:7595563 ]
  2. Xuan J, Pan G, Qiu Y, Yang L, Su M, Liu Y, Chen J, Feng G, Fang Y, Jia W, Xing Q, He L: Metabolomic profiling to identify potential serum biomarkers for schizophrenia and risperidone action. J Proteome Res. 2011 Dec 2;10(12):5433-43. doi: 10.1021/pr2006796. Epub 2011 Nov 8. [PubMed:22007635 ]
  3. Cai HL, Li HD, Yan XZ, Sun B, Zhang Q, Yan M, Zhang WY, Jiang P, Zhu RH, Liu YP, Fang PF, Xu P, Yuan HY, Zhang XH, Hu L, Yang W, Ye HS: Metabolomic analysis of biochemical changes in the plasma and urine of first-episode neuroleptic-naive schizophrenia patients after treatment with risperidone. J Proteome Res. 2012 Aug 3;11(8):4338-50. doi: 10.1021/pr300459d. Epub 2012 Jul 26. [PubMed:22800120 ]
Frontotemporal dementia
  1. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Glucoglycinuria
  1. KASER H, COTTIER P, ANTENER I: Glucoglycinuria, a new familial syndrome. J Pediatr. 1962 Sep;61:386-94. [PubMed:14454131 ]
Histidinemia
  1. Nyhan WL, Hilton S: Histidinuria: defective transport of histidine. Am J Med Genet. 1992 Nov 15;44(5):558-61. [PubMed:1481808 ]
Irritable bowel syndrome
  1. Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ]
Juvenile myoclonic 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 ]
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 ]
Lung Cancer
  1. Stretch C, Eastman T, Mandal R, Eisner R, Wishart DS, Mourtzakis M, Prado CM, Damaraju S, Ball RO, Greiner R, Baracos VE: Prediction of skeletal muscle and fat mass in patients with advanced cancer using a metabolomic approach. J Nutr. 2012 Jan;142(1):14-21. doi: 10.3945/jn.111.147751. Epub 2011 Dec 7. [PubMed:22157537 ]
Maple syrup urine disease
  1. Gronwald W, Klein MS, Kaspar H, Fagerer SR, Nurnberger N, Dettmer K, Bertsch T, Oefner PJ: Urinary metabolite quantification employing 2D NMR spectroscopy. Anal Chem. 2008 Dec 1;80(23):9288-97. doi: 10.1021/ac801627c. [PubMed:19551947 ]
Obesity
  1. Simone Wahl, Christina Holzapfel, Zhonghao Yu, Michaela Breier, Ivan Kondofersky, Christiane Fuchs, Paula Singmann, Cornelia Prehn, Jerzy Adamski, Harald Grallert, Thomas Illig, Rui Wang-Sattler, Thomas Reinehr (2013). Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children. Metabolomics.
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 ]
Phenylketonuria
  1. Gronwald W, Klein MS, Kaspar H, Fagerer SR, Nurnberger N, Dettmer K, Bertsch T, Oefner PJ: Urinary metabolite quantification employing 2D NMR spectroscopy. Anal Chem. 2008 Dec 1;80(23):9288-97. doi: 10.1021/ac801627c. [PubMed:19551947 ]
Propionic acidemia
  1. Gronwald W, Klein MS, Kaspar H, Fagerer SR, Nurnberger N, Dettmer K, Bertsch T, Oefner PJ: Urinary metabolite quantification employing 2D NMR spectroscopy. Anal Chem. 2008 Dec 1;80(23):9288-97. doi: 10.1021/ac801627c. [PubMed:19551947 ]
  2. Riemersma M, Hazebroek MR, Helderman-van den Enden ATJM, Salomons GS, Ferdinandusse S, Brouwers MCGJ, van der Ploeg L, Heymans S, Glatz JFC, van den Wijngaard A, Krapels IPC, Bierau J, Brunner HG: Propionic acidemia as a cause of adult-onset dilated cardiomyopathy. Eur J Hum Genet. 2017 Nov;25(11):1195-1201. doi: 10.1038/ejhg.2017.127. Epub 2017 Aug 30. [PubMed:28853722 ]
Refractory localization-related 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 ]
Sarcosinemia
  1. Sewell AC, Krille M, Wilhelm I: Sarcosinaemia in a retarded, amaurotic child. Eur J Pediatr. 1986 Feb;144(5):508-10. [PubMed:2420598 ]
  2. Benarrosh A, Garnotel R, Henry A, Arndt C, Gillery P, Motte J, Bakchine S: A young adult with sarcosinemia. No benefit from long duration treatment with memantine. JIMD Rep. 2013;9:93-96. doi: 10.1007/8904_2012_185. Epub 2012 Oct 21. [PubMed:23430553 ]
Tyrosinemia I
  1. Gronwald W, Klein MS, Kaspar H, Fagerer SR, Nurnberger N, Dettmer K, Bertsch T, Oefner PJ: Urinary metabolite quantification employing 2D NMR spectroscopy. Anal Chem. 2008 Dec 1;80(23):9288-97. doi: 10.1021/ac801627c. [PubMed:19551947 ]
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 ]
N-acetylglutamate synthetase deficiency
  1. Schubiger G, Bachmann C, Barben P, Colombo JP, Tonz O, Schupbach D: N-acetylglutamate synthetase deficiency: diagnosis, management and follow-up of a rare disorder of ammonia detoxication. Eur J Pediatr. 1991 Mar;150(5):353-6. [PubMed:2044610 ]
Iminoglycinuria
  1. Lasley L, Scriver CR: Ontogeny of amino acid reabsorption in human kidney. Evidence from the homozygous infant with familial renal iminoglycinuria for multiple proline and glycine systems. Pediatr Res. 1979 Jan;13(1):65-70. [PubMed:432003 ]
  2. Broer S, Bailey CG, Kowalczuk S, Ng C, Vanslambrouck JM, Rodgers H, Auray-Blais C, Cavanaugh JA, Broer A, Rasko JE: Iminoglycinuria and hyperglycinuria are discrete human phenotypes resulting from complex mutations in proline and glycine transporters. J Clin Invest. 2008 Dec;118(12):3881-92. doi: 10.1172/JCI36625. Epub 2008 Nov 6. [PubMed:19033659 ]
  3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Gout
  1. Shao T, Shao L, Li H, Xie Z, He Z, Wen C: Combined Signature of the Fecal Microbiome and Metabolome in Patients with Gout. Front Microbiol. 2017 Feb 21;8:268. doi: 10.3389/fmicb.2017.00268. eCollection 2017. [PubMed:28270806 ]
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. .
Nonketotic Hyperglycinemia
  1. Applegarth DA, Toone JR: Nonketotic hyperglycinemia (glycine encephalopathy): laboratory diagnosis. Mol Genet Metab. 2001 Sep-Oct;74(1-2):139-46. [PubMed:11592811 ]
D-Glyceric acidura
  1. Swanson MA, Garcia SM, Spector E, Kronquist K, Creadon-Swindell G, Walter M, Christensen E, Van Hove JLK, Sass JO: d-Glyceric aciduria does not cause nonketotic hyperglycinemia: A historic co-occurrence. Mol Genet Metab. 2017 Jun;121(2):80-82. doi: 10.1016/j.ymgme.2017.04.009. Epub 2017 Apr 20. [PubMed:28462797 ]
  2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Epilepsy, early-onset, vitamin B6-dependent
  1. Darin N, Reid E, Prunetti L, Samuelsson L, Husain RA, Wilson M, El Yacoubi B, Footitt E, Chong WK, Wilson LC, Prunty H, Pope S, Heales S, Lascelles K, Champion M, Wassmer E, Veggiotti P, de Crecy-Lagard V, Mills PB, Clayton PT: Mutations in PROSC Disrupt Cellular Pyridoxal Phosphate Homeostasis and Cause Vitamin-B6-Dependent Epilepsy. Am J Hum Genet. 2016 Dec 1;99(6):1325-1337. doi: 10.1016/j.ajhg.2016.10.011. [PubMed:27912044 ]
Hyperglycinemia, lactic acidosis, and seizures
  1. Mayr JA, Zimmermann FA, Fauth C, Bergheim C, Meierhofer D, Radmayr D, Zschocke J, Koch J, Sperl W: Lipoic acid synthetase deficiency causes neonatal-onset epilepsy, defective mitochondrial energy metabolism, and glycine elevation. Am J Hum Genet. 2011 Dec 9;89(6):792-7. doi: 10.1016/j.ajhg.2011.11.011. [PubMed:22152680 ]
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 ]
Neu-Laxova Syndrome 1
  1. Shaheen R, Rahbeeni Z, Alhashem A, Faqeih E, Zhao Q, Xiong Y, Almoisheer A, Al-Qattan SM, Almadani HA, Al-Onazi N, Al-Baqawi BS, Saleh MA, Alkuraya FS: Neu-Laxova syndrome, an inborn error of serine metabolism, is caused by mutations in PHGDH. Am J Hum Genet. 2014 Jun 5;94(6):898-904. doi: 10.1016/j.ajhg.2014.04.015. Epub 2014 May 15. [PubMed:24836451 ]
  2. El-Hattab AW, Shaheen R, Hertecant J, Galadari HI, Albaqawi BS, Nabil A, Alkuraya FS: On the phenotypic spectrum of serine biosynthesis defects. J Inherit Metab Dis. 2016 May;39(3):373-381. doi: 10.1007/s10545-016-9921-5. Epub 2016 Mar 10. [PubMed:26960553 ]
Phosphoserine Aminotransferase Deficiency
  1. Hart CE, Race V, Achouri Y, Wiame E, Sharrard M, Olpin SE, Watkinson J, Bonham JR, Jaeken J, Matthijs G, Van Schaftingen E: Phosphoserine aminotransferase deficiency: a novel disorder of the serine biosynthesis pathway. Am J Hum Genet. 2007 May;80(5):931-7. Epub 2007 Mar 30. [PubMed:17436247 ]
Phosphoserine Phosphatase Deficiency
  1. Jaeken J, Detheux M, Van Maldergem L, Frijns JP, Alliet P, Foulon M, Carchon H, Van Schaftingen E: 3-Phosphoglycerate dehydrogenase deficiency and 3-phosphoserine phosphatase deficiency: inborn errors of serine biosynthesis. J Inherit Metab Dis. 1996;19(2):223-6. [PubMed:8739971 ]
Pyridoxamine 5-prime-phosphate oxidase deficiency
  1. Plecko B, Paul K, Paschke E, Stoeckler-Ipsiroglu S, Struys E, Jakobs C, Hartmann H, Luecke T, di Capua M, Korenke C, Hikel C, Reutershahn E, Freilinger M, Baumeister F, Bosch F, Erwa W: Biochemical and molecular characterization of 18 patients with pyridoxine-dependent epilepsy and mutations of the antiquitin (ALDH7A1) gene. Hum Mutat. 2007 Jan;28(1):19-26. [PubMed:17068770 ]
Ulcerative colitis
  1. Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
Early preeclampsia
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
Late-onset preeclampsia
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ]
Lewy body disease
  1. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Associated OMIM IDs
DrugBank IDDB00145
Phenol Explorer Compound IDNot Available
FoodDB IDFDB000484
KNApSAcK IDC00001361
Chemspider ID730
KEGG Compound IDC00037
BioCyc IDGLY
BiGG ID33610
Wikipedia LinkGlycine
METLIN ID20
PubChem Compound750
PDB IDGLY
ChEBI ID15428
References
Synthesis Reference Anslow, Winston K.; King, Harold. Synthesis of glycine. Journal of the Chemical Society (1929), 2163-6.
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. doi: 10.1038/nature07762. [PubMed: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. [PubMed:2026685 ]
  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. 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 ]
  5. Bales JR, Higham DP, Howe I, Nicholson JK, Sadler PJ: Use of high-resolution proton nuclear magnetic resonance spectroscopy for rapid multi-component analysis of urine. Clin Chem. 1984 Mar;30(3):426-32. [PubMed:6321058 ]
  6. 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. [PubMed:12834252 ]
  7. Collins JW, Macdermott S, Bradbrook RA, Keeley FX Jr, Timoney AG: Is using ethanol-glycine irrigating fluid monitoring and 'good surgical practice' enough to prevent harmful absorption during transurethral resection of the prostate? BJU Int. 2006 Jun;97(6):1247-51. [PubMed:16686720 ]
  8. 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. [PubMed:6198473 ]
  9. Christie GR, Ford D, Howard A, Clark MA, Hirst BH: Glycine supply to human enterocytes mediated by high-affinity basolateral GLYT1. Gastroenterology. 2001 Feb;120(2):439-48. [PubMed:11159884 ]
  10. Jones CM, Smith M, Henderson MJ: Reference data for cerebrospinal fluid and the utility of amino acid measurement for the diagnosis of inborn errors of metabolism. Ann Clin Biochem. 2006 Jan;43(Pt 1):63-6. [PubMed:16390611 ]
  11. 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 ]
  12. 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 ]
  13. 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 ]
  14. Bennett FI, Jackson AA: Glycine is not formed through the amino transferase reaction in human or rat placenta. Placenta. 1998 May;19(4):329-31. [PubMed:9639330 ]
  15. Gomeza J, Ohno K, Hulsmann S, Armsen W, Eulenburg V, Richter DW, Laube B, Betz H: Deletion of the mouse glycine transporter 2 results in a hyperekplexia phenotype and postnatal lethality. Neuron. 2003 Nov 13;40(4):797-806. [PubMed:14622583 ]
  16. Boneh A, Degani Y, Harari M: Prognostic clues and outcome of early treatment of nonketotic hyperglycinemia. Pediatr Neurol. 1996 Sep;15(2):137-41. [PubMed:8888048 ]
  17. Dicke JM, Verges D, Kelley LK, Smith CH: Glycine uptake by microvillous and basal plasma membrane vesicles from term human placentae. Placenta. 1993 Jan-Feb;14(1):85-92. [PubMed:8456092 ]
  18. Prescot AP, de B Frederick B, Wang L, Brown J, Jensen JE, Kaufman MJ, Renshaw PF: In vivo detection of brain glycine with echo-time-averaged (1)H magnetic resonance spectroscopy at 4.0 T. Magn Reson Med. 2006 Mar;55(3):681-6. [PubMed:16453318 ]
  19. Byard RW, Harrison R, Wells R, Gilbert JD: Glycine toxicity and unexpected intra-operative death. J Forensic Sci. 2001 Sep;46(5):1244-6. [PubMed:11569574 ]
  20. Khan SA, Cox IJ, Hamilton G, Thomas HC, Taylor-Robinson SD: In vivo and in vitro nuclear magnetic resonance spectroscopy as a tool for investigating hepatobiliary disease: a review of H and P MRS applications. Liver Int. 2005 Apr;25(2):273-81. [PubMed:15780050 ]
  21. Van Hove JL, Vande Kerckhove K, Hennermann JB, Mahieu V, Declercq P, Mertens S, De Becker M, Kishnani PS, Jaeken J: Benzoate treatment and the glycine index in nonketotic hyperglycinaemia. J Inherit Metab Dis. 2005;28(5):651-63. [PubMed:16151895 ]

Only showing the first 10 proteins. There are 41 proteins in total.

Enzymes

General function:
Involved in 5-aminolevulinate synthase activity
Specific function:
Not Available
Gene Name:
ALAS2
Uniprot ID:
P22557
Molecular weight:
64632.86
Reactions
Succinyl-CoA + Glycine → 5-Aminolevulinic acid + Coenzyme A + CO(2)details
Succinyl-CoA + Glycine → 5-Aminolevulinic acid + Coenzyme A + Carbon dioxidedetails
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. Hungerer C, Troup B, Romling U, Jahn D: Regulation of the hemA gene during 5-aminolevulinic acid formation in Pseudomonas aeruginosa. J Bacteriol. 1995 Mar;177(6):1435-43. [PubMed:7883699 ]
  4. Shoolingin-Jordan PM, Al-Daihan S, Alexeev D, Baxter RL, Bottomley SS, Kahari ID, Roy I, Sarwar M, Sawyer L, Wang SF: 5-Aminolevulinic acid synthase: mechanism, mutations and medicine. Biochim Biophys Acta. 2003 Apr 11;1647(1-2):361-6. [PubMed:12686158 ]
  5. Munakata H, Yamagami T, Nagai T, Yamamoto M, Hayashi N: Purification and structure of rat erythroid-specific delta-aminolevulinate synthase. J Biochem. 1993 Jul;114(1):103-11. [PubMed:8407861 ]
General function:
Involved in 5-aminolevulinate synthase activity
Specific function:
Not Available
Gene Name:
ALAS1
Uniprot ID:
P13196
Molecular weight:
70580.325
Reactions
Succinyl-CoA + Glycine → 5-Aminolevulinic acid + Coenzyme A + CO(2)details
Succinyl-CoA + Glycine → 5-Aminolevulinic acid + Coenzyme A + Carbon dioxidedetails
References
  1. Turbeville TD, Zhang J, Hunter GA, Ferreira GC: Histidine 282 in 5-aminolevulinate synthase affects substrate binding and catalysis. Biochemistry. 2007 May 22;46(20):5972-81. Epub 2007 May 1. [PubMed:17469798 ]
  2. He XM, Zhou J, Cheng Y, Fan J: [Purification and production of the extracellular 5-aminolevulinate from recombiniant Escherichia coli expressing yeast ALAS]. Sheng Wu Gong Cheng Xue Bao. 2007 May;23(3):520-4. [PubMed:17578005 ]
General function:
Involved in glycine C-acetyltransferase activity
Specific function:
Not Available
Gene Name:
GCAT
Uniprot ID:
O75600
Molecular weight:
47973.79
Reactions
Acetyl-CoA + Glycine → Coenzyme A + L-2-Amino-3-oxobutanoic aciddetails
Acetyl-CoA + Glycine → Coenzyme A + L-2-Amino-3-oxobutanoic aciddetails
References
  1. Bashir Q, Rashid N, Akhtar M: Mechanism and substrate stereochemistry of 2-amino-3-oxobutyrate CoA ligase: implications for 5-aminolevulinate synthase and related enzymes. Chem Commun (Camb). 2006 Dec 28;(48):5065-7. Epub 2006 Oct 13. [PubMed:17146529 ]
General function:
Involved in oxidoreductase activity
Specific function:
Metabolizes sarcosine, L-pipecolic acid and L-proline.
Gene Name:
PIPOX
Uniprot ID:
Q9P0Z9
Molecular weight:
44065.515
Reactions
Sarcosine + Water + Oxygen → Glycine + Formaldehyde + Hydrogen peroxidedetails
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 ]
General function:
Involved in D-amino-acid oxidase activity
Specific function:
Regulates the level of the neuromodulator D-serine in the brain. Has high activity towards D-DOPA and contributes to dopamine synthesis. Could act as a detoxifying agent which removes D-amino acids accumulated during aging. Acts on a variety of D-amino acids with a preference for those having small hydrophobic side chains followed by those bearing polar, aromatic, and basic groups. Does not act on acidic amino acids.
Gene Name:
DAO
Uniprot ID:
P14920
Molecular weight:
39473.75
Reactions
Glycine + Water + Oxygen → Glyoxylic acid + Ammonia + Hydrogen peroxidedetails
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
GART
Uniprot ID:
P22102
Molecular weight:
107766.295
Reactions
Adenosine triphosphate + 5-Phosphoribosylamine + Glycine → ADP + Phosphoric acid + Glycineamideribotidedetails
Adenosine triphosphate + 5-Phosphoribosylamine + Glycine → ADP + Phosphoric acid + Glycineamideribotidedetails
General function:
Involved in transaminase activity
Specific function:
Can metabolize asymmetric dimethylarginine (ADMA) via transamination to alpha-keto-delta-(NN-dimethylguanidino) valeric acid (DMGV). ADMA is a potent inhibitor of nitric-oxide (NO) synthase, and this activity provides mechanism through which the kidney regulates blood pressure.
Gene Name:
AGXT2
Uniprot ID:
Q9BYV1
Molecular weight:
57155.905
Reactions
L-Alanine + Glyoxylic acid → Pyruvic acid + Glycinedetails
Glycine + Oxoglutaric acid → Glyoxylic acid + 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. Baker PR, Cramer SD, Kennedy M, Assimos DG, Holmes RP: Glycolate and glyoxylate metabolism in HepG2 cells. Am J Physiol Cell Physiol. 2004 Nov;287(5):C1359-65. Epub 2004 Jul 7. [PubMed:15240345 ]
  4. Takada Y, Mori T, Noguchi T: The effect of vitamin B6 deficiency on alanine: glyoxylate aminotransferase isoenzymes in rat liver. Arch Biochem Biophys. 1984 Feb 15;229(1):1-6. [PubMed:6703688 ]
General function:
Involved in folic acid binding
Specific function:
Catalyzes the methylation of glycine by using S-adenosylmethionine (AdoMet) to form N-methylglycine (sarcosine) with the concomitant production of S-adenosylhomocysteine (AdoHcy). Possible crucial role in the regulation of tissue concentration of AdoMet and of metabolism of methionine.
Gene Name:
GNMT
Uniprot ID:
Q14749
Molecular weight:
32742.0
Reactions
S-Adenosylmethionine + Glycine → S-Adenosylhomocysteine + Sarcosinedetails
References
  1. Soriano A, Castillo R, Christov C, Andres J, Moliner V, Tunon I: Catalysis in glycine N-methyltransferase: testing the electrostatic stabilization and compression hypothesis. Biochemistry. 2006 Dec 19;45(50):14917-25. [PubMed:17154529 ]
  2. Luka Z, Pakhomova S, Loukachevitch LV, Egli M, Newcomer ME, Wagner C: 5-methyltetrahydrofolate is bound in intersubunit areas of rat liver folate-binding protein glycine N-methyltransferase. J Biol Chem. 2007 Feb 9;282(6):4069-75. Epub 2006 Dec 7. [PubMed:17158459 ]
  3. Velichkova P, Himo F: Methyl transfer in glycine N-methyltransferase. A theoretical study. J Phys Chem B. 2005 Apr 28;109(16):8216-9. [PubMed:16851960 ]
General function:
Involved in proteolysis
Specific function:
Broad specificity aminopeptidase. Plays a role in the final digestion of peptides generated from hydrolysis of proteins by gastric and pancreatic proteases. May play a critical role in the pathogenesis of cholesterol gallstone disease. May be involved in the metabolism of regulatory peptides of diverse cell types, responsible for the processing of peptide hormones, such as angiotensin III and IV, neuropeptides, and chemokines. Found to cleave antigen peptides bound to major histocompatibility complex class II molecules of presenting cells and to degrade neurotransmitters at synaptic junctions. Is also implicated as a regulator of IL-8 bioavailability in the endometrium, and therefore may contribute to the regulation of angiogenesis. Is used as a marker for acute myeloid leukemia and plays a role in tumor invasion. In case of human coronavirus 229E (HCoV-229E) infection, serves as receptor for HCoV-229E spike glycoprotein. Mediates as well human cytomegalovirus (HCMV) infection.
Gene Name:
ANPEP
Uniprot ID:
P15144
Molecular weight:
Not Available
Reactions
Cysteinylglycine + Water → L-Cysteine + Glycinedetails
R-S-Cysteinylglycine + Water → S-Substituted L-cysteine + Glycinedetails
General function:
Amino acid transport and metabolism
Specific function:
Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development. May be involved in the response to heart failure by elevating local creatine synthesis.
Gene Name:
GATM
Uniprot ID:
P50440
Molecular weight:
48455.01
Reactions
L-Arginine + Glycine → Ornithine + Guanidoacetic aciddetails
References
  1. Wang L, Zhang Y, Shao M, Zhang H: Spatiotemporal expression of the creatine metabolism related genes agat, gamt and ct1 during zebrafish embryogenesis. Int J Dev Biol. 2007;51(3):247-53. [PubMed:17486546 ]
  2. Dutta U, Cohenford MA, Guha M, Dain JA: Non-enzymatic interactions of glyoxylate with lysine, arginine, and glucosamine: a study of advanced non-enzymatic glycation like compounds. Bioorg Chem. 2007 Feb;35(1):11-24. Epub 2006 Sep 12. [PubMed:16970975 ]

Transporters

General function:
Amino acid transport and metabolism
Specific function:
Neutral amino acid/proton symporter. Has a pH-dependent electrogenic transport activity for small amino acids such as glycine, alanine and proline. Besides small apolar L-amino acids, it also recognize their D-enantiomers and selected amino acid derivatives such as gamma-aminobutyric acid
Gene Name:
SLC36A1
Uniprot ID:
Q7Z2H8
Molecular weight:
53075.0
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. Bermingham JR Jr, Pennington J: Organization and expression of the SLC36 cluster of amino acid transporter genes. Mamm Genome. 2004 Feb;15(2):114-25. [PubMed:15058382 ]
  4. Broer A, Cavanaugh JA, Rasko JE, Broer S: The molecular basis of neutral aminoacidurias. Pflugers Arch. 2006 Jan;451(4):511-7. Epub 2005 Jul 29. [PubMed:16052352 ]
  5. Metzner L, Kalbitz J, Brandsch M: Transport of pharmacologically active proline derivatives by the human proton-coupled amino acid transporter hPAT1. J Pharmacol Exp Ther. 2004 Apr;309(1):28-35. Epub 2004 Jan 12. [PubMed:14718599 ]
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 41 proteins in total.