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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-05-20 20:40:41 UTC
HMDB IDHMDB0000517
Secondary Accession Numbers
  • HMDB00517
Metabolite Identification
Common NameL-Arginine
DescriptionArginine is an essential amino acid that is physiologically active in the L-form. In mammals, arginine is formally classified as a semi-essential or conditionally essential amino acid, depending on the developmental stage and health status of the individual. Infants are unable to effectively synthesize arginine, making it nutritionally essential for infants. Adults, however, are able to synthesize arginine in the urea cycle. Arginine can be considered to be a basic amino acid as the part of the side chain nearest to the backbone is long, carbon-containing, and hydrophobic, whereas the end of the side chain is a complex guanidinium group. With a pKa of 12.48, the guanidinium group is positively charged in neutral, acidic, and even most basic environments. Because of the conjugation between the double bond and the nitrogen lone pairs, the positive charge is delocalized. This group is able to form multiple H-bonds. L-Arginine is an amino acid that has numerous functions in the body. It helps dispose of ammonia, is used to make compounds such as nitric oxide, creatine, L-glutamate, and L-proline, and it can be converted into glucose and glycogen if needed. In large doses, L-arginine also stimulates the release of the hormones growth hormone and prolactin. Arginine is a known inducer of mTOR (mammalian target of rapamycin) and is responsible for inducing protein synthesis through the mTOR pathway. mTOR inhibition by rapamycin partially reduces arginine-induced protein synthesis (PMID: 20841502 ). Catabolic disease states such as sepsis, injury, and cancer cause an increase in arginine utilization, which can exceed normal body production, leading to arginine depletion. Arginine also activates AMP kinase (AMPK) which then stimulates skeletal muscle fatty acid oxidation and muscle glucose uptake, thereby increasing insulin secretion by pancreatic beta-cells (PMID: 21311355 ). Arginine is found in plant and animal proteins, such as dairy products, meat, poultry, fish, and nuts. The ratio of L-arginine to lysine is also important: soy and other plant proteins have more L-arginine than animal sources of protein.
Structure
Thumb
Synonyms
ValueSource
(2S)-2-amino-5-(carbamimidamido)Pentanoic acidChEBI
(2S)-2-amino-5-Guanidinopentanoic acidChEBI
(S)-2-amino-5-Guanidinopentanoic acidChEBI
(S)-2-amino-5-Guanidinovaleric acidChEBI
ArgChEBI
ArginineChEBI
L-(+)-ArginineChEBI
L-ArgChEBI
L-ArgininChEBI
RChEBI
(2S)-2-amino-5-(carbamimidamido)PentanoateGenerator
(2S)-2-amino-5-GuanidinopentanoateGenerator
(S)-2-amino-5-GuanidinopentanoateGenerator
(S)-2-amino-5-GuanidinovalerateGenerator
(S)-2-amino-5-[(Aminoiminomethyl)amino]-pentanoateHMDB
(S)-2-amino-5-[(Aminoiminomethyl)amino]-pentanoic acidHMDB
(S)-2-amino-5-[(Aminoiminomethyl)amino]pentanoateHMDB
(S)-2-amino-5-[(Aminoiminomethyl)amino]pentanoic acidHMDB
2-amino-5-GuanidinovalerateHMDB
2-amino-5-Guanidinovaleric acidHMDB
5-[(Aminoiminomethyl)amino]-L-norvalineHMDB
L-a-amino-D-GuanidinovalerateHMDB
L-a-amino-D-Guanidinovaleric acidHMDB
L-alpha-amino-delta-GuanidinovalerateHMDB
L-alpha-amino-delta-Guanidinovaleric acidHMDB
N5-(Aminoiminomethyl)-L-ornithineHMDB
DL-Arginine acetate, monohydrateMeSH
L-Isomer arginineMeSH
Monohydrate DL-arginine acetateMeSH
L ArginineMeSH
Arginine, L isomerMeSH
Arginine, L-isomerMeSH
Hydrochloride, arginineMeSH
Arginine hydrochlorideMeSH
DL Arginine acetate, monohydrateMeSH
Chemical FormulaC6H14N4O2
Average Molecular Weight174.201
Monoisotopic Molecular Weight174.111675712
IUPAC Name(2S)-2-amino-5-carbamimidamidopentanoic acid
Traditional NameL-arginine
CAS Registry Number74-79-3
SMILES
N[C@@H](CCCNC(N)=N)C(O)=O
InChI Identifier
InChI=1S/C6H14N4O2/c7-4(5(11)12)2-1-3-10-6(8)9/h4H,1-3,7H2,(H,11,12)(H4,8,9,10)/t4-/m0/s1
InChI KeyODKSFYDXXFIFQN-BYPYZUCNSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as l-alpha-amino acids. These are alpha amino acids which have the L-configuration of the alpha-carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentL-alpha-amino acids
Alternative Parents
Substituents
  • L-alpha-amino acid
  • Fatty acid
  • Guanidine
  • Amino acid
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Carboximidamide
  • Propargyl-type 1,3-dipolar organic compound
  • Organic 1,3-dipolar compound
  • Amine
  • Hydrocarbon derivative
  • Organic oxide
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Organopnictogen compound
  • Primary aliphatic amine
  • Organic oxygen compound
  • Organic nitrogen compound
  • Carbonyl group
  • 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

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point222 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility182 mg/mL at 25 °CHuman Metabolome Project
LogP-4.20HANSCH,C ET AL. (1995)
Predicted Properties
PropertyValueSource
Water Solubility7.59 g/LALOGPS
logP-3.5ALOGPS
logP-3.2ChemAxon
logS-1.9ALOGPS
pKa (Strongest Acidic)2.41ChemAxon
pKa (Strongest Basic)12.41ChemAxon
Physiological Charge1ChemAxon
Hydrogen Acceptor Count6ChemAxon
Hydrogen Donor Count5ChemAxon
Polar Surface Area125.22 ŲChemAxon
Rotatable Bond Count5ChemAxon
Refractivity53.92 m³·mol⁻¹ChemAxon
Polarizability17.8 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)splash10-0a4i-1910000000-0191c1a63652c493660bView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-00di-9810000000-eb6eb73302b678cf0a24View in MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-00di-9700000000-e47b41cff0e873f53932View in MoNA
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-0a4i-1920000000-8ae5af11398835d26bedView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0a4i-1910000000-0191c1a63652c493660bView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00di-9810000000-eb6eb73302b678cf0a24View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00di-9700000000-e47b41cff0e873f53932View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0a4i-1920000000-8ae5af11398835d26bedView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0udi-0900000000-afc3ca93f8fbf54ec9f4View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-00dl-9100000000-60db59de76b9a9b5bfc8View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-004i-8900000000-f47c4f9480612fdbc962View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-0fk9-0946231100-3d57d2304dcb33feab3fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-000i-0900000000-5b6dd6fb263ea09289fcView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-001i-0900000000-dfe35b3438d19320d8cbView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-00di-0900000000-f666ab7e5354bce67a2eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-03kd-0977452210-046c4b70bd0ec351c41dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-014i-9000000000-2f51e43e530976d63633View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-001i-0900000000-b9ebb7ebccee1a313888View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-00di-0900000000-5379b6fb6ea2313101f9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-001i-0900000000-835751d54af24bd337e7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-001i-0900000000-2d2b5fd7617ccb227bb1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT , negativesplash10-001i-0900000000-dfe35b3438d19320d8cbView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT , negativesplash10-00di-0900000000-f666ab7e5354bce67a2eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT , negativesplash10-001i-0900000000-b9ebb7ebccee1a313888View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT , negativesplash10-00di-0900000000-5379b6fb6ea2313101f9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , negativesplash10-001i-0900000000-835751d54af24bd337e7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , negativesplash10-001i-0900000000-2d2b5fd7617ccb227bb1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-00di-3900000000-8c82418f7b35a97fb9b3View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00di-9000000000-19b62da79866318c52ddView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-00di-9000000000-64b046d21bdcbb8d5923View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-004i-0900000000-e07b937b6867d1f62293View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0a4i-0900000000-87ab853583aab2973cfbView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0a4i-2900000000-28814246b728a51e761cView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0a4i-2900000000-cfe7e406be2172e94fbaView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-004i-0910000000-478a8345915bc15d85b5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-0a4i-2900000000-c930c47ecbe6975a00fcView in MoNA
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H 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
  • Cytoplasm
  • Extracellular
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Sweat
  • Urine
Tissue Location
  • Adipose Tissue
  • Adrenal Cortex
  • Bladder
  • Epidermis
  • Fibroblasts
  • Gonads
  • Intestine
  • Kidney
  • Liver
  • Muscle
  • Myelin
  • Nerve Cells
  • Neuron
  • Pancreas
  • Placenta
  • Platelet
  • Prostate
  • Skeletal Muscle
  • Spleen
  • Stratum Corneum
  • Testes
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified129.5 +/- 30 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified10-66 uMChildren (1-13 years old)BothNormal details
BloodDetected and Quantified101 (72.5-112) uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified14-140 uMChildren (1 - 13 years old)BothNormal details
BloodDetected and Quantified6-140 uMChildren (1 - 13 years old)BothNormal details
BloodDetected and Quantified80 +/- 60 uMInfant (0-1 year old)BothNormal details
BloodDetected and Quantified99.00 +/- 22.8 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified23-86 uMAdult (>18 years old)Not SpecifiedNormal
    • Lysinuric Protein...
details
BloodDetected and Quantified50-100 uMChildren (1-13 years old)MaleNormal details
BloodDetected and Quantified80 (60.0 - 100) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified26-155 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified12-112 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected but not Quantified Adult (>18 years old)Both
Normal
details
BloodDetected and Quantified50.0 +/- 20.0 uMNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified92.0 +/- 19.0 uMChildren (1-13 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified84.0 +/- 20.0 uMChildren (1-13 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified89.0 +/- 26.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified75.0 +/- 24.0 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified81.4 +/- 19.3 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified110.11(21.24) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified75 +/- 24 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified87.4(71.4-103.9) uMChildren (1-13 uears old)Both
Normal
details
BloodDetected and Quantified77 +/- 20 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified28-99 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified76.6 +/- 20.9 uMChildren (1-13 years old)MaleNormal details
BloodDetected and Quantified111.6 (82.2-140.9) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified40-160 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified89 +/- 26 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified40.00-130.0 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified113.6 +/- 14.6 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified218.0 +/- 6.0 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified63.45 (40.0 - 86.9) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified17-119 uMNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified21-99 uMInfant (1 - 3 months old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified32-142 uMChildren (3 months - 6 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified44-130 uMChildren (6 - 18 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified9.33 +/- 0.81 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified20.2 +/- 6.3 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified24.2 +/- 5.1 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified30-Oct uMChildren (1-13 years old)MaleNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified18.3 +/- 3.2 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified6000-29000 uMNot SpecifiedNot SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified20.5 (15.7 - 25.3) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified17.9 +/- 5.7 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified24.7 (18.3-31.1) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified13-35 uMChildren (1-13 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified8-29.4 uMChildren (0 - 10 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified6.8-32.6 uMAdolescent (>11 years old)Both
Normal
    • BC Children's Hos...
details
FecesDetected but not Quantified Not SpecifiedBoth
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected and Quantified2659.763 (1530.799-3788.727) nmol/g wet fecesInfant (0-1 year old)Not Specified
Normal
details
FecesDetected and Quantified4898.556 (2353.603-7443.509) nmol/g wet fecesInfant (0-1 year old)Not Specified
Normal
details
FecesDetected and Quantified7003.404 (4209.696-9797.112) nmol/g wet fecesInfant (0-1 year old)Not Specified
Normal
details
FecesDetected but not Quantified Children (1-13 years old)BothNormal 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 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)FemaleNormal details
FecesDetected and Quantified60 +/- 70 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedNormal details
SalivaDetected and Quantified24.89 +/- 6.78 uMAdult (>18 years old)FemaleNormal details
SalivaDetected and Quantified9.00 +/- 2.75 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
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)Not SpecifiedNormal details
SalivaDetected and Quantified6.5 +/- 1.6 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified5.2 +/- 1.7 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified7.5 +/- 2.3 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified28.9 +/- 20.1 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified35.73 +/- 17.33 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected and Quantified5.9 +/- 1.4 uMAdult (>18 years old)Male
Normal
details
SweatDetected and Quantified< 10 uMAdult (60 years old)Male
Normal
details
SweatDetected and Quantified< 10 uMAdult (40 years old)Male
Normal
details
UrineDetected and Quantified2.37 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified1-6 umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected but not Quantified Not AvailableBothNormal details
UrineDetected and Quantified0-7 umol/mmol creatinineChildren (1 - 13 years old)BothNormal details
UrineDetected and Quantified1.363 +/- 0.584 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified<4.02-22.97 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
details
UrineDetected and Quantified0 - 17.64 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 14.02 umol/mmol creatinineInfant (1 - 6 months old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 14.02 umol/mmol creatinineInfant (6 months - <1 year old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified16.69 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Shaykhutdinov RA,...
details
UrineDetected and Quantified0 - 10.51 umol/mmol creatinineChildren (1 - 2 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 11.76 umol/mmol creatinineChildren (2 - 4 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 9.38 umol/mmol creatinineChildren (4 - 13 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 6.90 umol/mmol creatinineAdolescent (13 - 21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 6.90 umol/mmol creatinineAdult (>21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0.83 +/- 0.43 umol/mmol creatinineChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified2.0 +/- 1.15 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified1.35 +/- 0.89 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
details
UrineDetected and Quantified1.367 +/- 0.907 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified2.0133 +/- 1.167 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified3.5 (2.0-6.0) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified35.855 +/- 25.427 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified1.96 +/- 1.77 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified7.4 (3.2-14.6) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified1.363 +/- 0.584 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified8.6 (3.8-18.6) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified16.2711 +/- 7.2472 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified2.0 (1.5-2.5) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified1.515 (0.395 - 2.632) umol/mmol creatinineAdult (>18 years old)BothNormal
    details
    Abnormal Concentrations
    BiospecimenStatusValueAgeSexConditionReferenceDetails
    BloodDetected and Quantified68-422 uMNewborn (0-30 days old)Not SpecifiedArgininemia details
    BloodDetected and Quantified125.6 (37.3) uMAdult (>18 years old)FemaleDown syndrome pregnancy details
    BloodDetected and Quantified132.7 (50.7) uMAdult (>18 years old)FemalePregnancy details
    BloodDetected and Quantified73.0 (6.0-140.0) uMAdult (>18 years old)BothArgininemia
      • MetaGene: Metabol...
    details
    BloodDetected and Quantified<1-17 uMChildren (1 - 13 years old)BothLysinuric protein intolerance details
    BloodDetected and Quantified19-103 uMNewborn (0-30 days old)MaleN-Acetylglutamate synthetase deficiency details
    BloodDetected and Quantified35-178 uMInfant (0-1 year old)Male
    N-Acetylglutamate synthetase deficiency
    details
    BloodDetected and Quantified44.500-256 uMChildren (1-13 years old)Male
    N-acetylglutamate synthetase deficiency
    details
    BloodDetected and Quantified45.2 (33.2-63.4) uMAdult (>18 years old)Both
    Lysinuric protein intolerance
    details
    BloodDetected and Quantified6.8-94.4 uMChildren (1 - 13 years old)BothCitrullinemia Type I details
    BloodDetected and Quantified101.6 +/- 39.2 uMAdult (>18 years old)BothChronic renal failure details
    BloodDetected and Quantified27 (12-58) uMAdult (>18 years old)Not SpecifiedLysinuric protein intolerance
      • Lysinuric Protein...
    details
    BloodDetected but not Quantified Adult (>18 years old)BothColorectal cancer details
    BloodDetected and Quantified110.97 +/- 26.59 uMAdult (>18 years old)Female
    Pregnancy with fetuses with trisomy 18
    details
    BloodDetected and Quantified122.82 +/- 39.7 uMAdult (>18 years old)FemalePregnancy details
    BloodDetected and Quantified113.4 (16.5) uMAdult (>18 years old)FemaleEarly preeclampsia details
    BloodDetected and Quantified124.2 (28.8) uMAdult (>18 years old)FemalePregnancy details
    BloodDetected and Quantified45 (23.0 - 67.0) uMAdult (>18 years old)BothPermanent Intestinal Failure details
    BloodDetected and Quantified99 uMNewborn (0-30 days old)Male
    Narp Syndrome
    details
    BloodDetected and Quantified24-47 uMChildren (1-13 years old)MaleNarp Syndrome details
    BloodDetected and Quantified18-156 uMInfant (0-1 year old)BothNarp Syndrome details
    BloodDetected and Quantified12-59 uMChildren (1-13 years old)BothPhosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic details
    BloodDetected and Quantified10 uMNewborn (0-30 days old)MalePhosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic details
    BloodDetected but not Quantified Adult (>18 years old)Both
    Schizophrenia
    details
    BloodDetected and Quantified82.6(67.7-97.9) uMChildren (1-13 uears old)Both
    Environmental enteric dysfunction
    details
    BloodDetected and Quantified125.77(25.5) uMAdult (>18 years old)BothHeart failure with preserved ejection fraction details
    BloodDetected and Quantified118.19(17.47) uMAdult (>18 years old)BothHeart failure with reduced ejection fraction details
    BloodDetected and Quantified136.3 (55.5) uMAdult (>18 years old)FemaleLate-onset preeclampsia details
    BloodDetected and Quantified131.2 (35.9) uMAdult (>18 years old)FemalePregnancy details
    BloodDetected and Quantified91.34 +/- 13.1 uMElderly (>65 years old)BothAlzheimer's disease details
    BloodDetected and Quantified38 (11-121) uMChildren (1-13 years old)Female
    Pearson Syndrome
    details
    BloodDetected and Quantified148-315 uMNewborn (0-30 days old)BothCitrullinemia type II, neonatal-onset details
    BloodDetected and Quantified35 +/- 12 uMChildren (1-13 years old)BothCutis laxa, autosomal recessive, type IIIA details
    BloodDetected and Quantified75.9 +/- 15.7 uMChildren (1-13 years old)MaleDuchenne Muscular Dystrophy details
    BloodDetected and Quantified30 uMChildren (1-13 years old)MalePearson Syndrome details
    BloodDetected and Quantified108.0 +/- 21.6 uMChildren (1-13 years old)Both
    Obesity
      • Metabolomics reve...
    details
    BloodDetected and Quantified117.9 +/- 20.1 uMChildren (1-13 years old)Both
    Obesity
      • Metabolomics reve...
    details
    BloodDetected and Quantified1250.0 (1000.0-1500.0) uMChildren (1-13 years old)BothArgininemia
      • MetaGene: Metabol...
    details
    BloodDetected and Quantified218.0 +/- 7.0 uMAdult (>18 years old)Both
    Heart failure
    details
    BloodDetected and Quantified907 uMChildren (1-13 years old)MaleArgininemia details
    BloodDetected and Quantified252-1579.200 uMChildren (1-13 years old)FemaleArgininemia details
    Cerebrospinal Fluid (CSF)Detected and Quantified88000 uMChildren (1-13 years old)Femaleargininemia details
    Cerebrospinal Fluid (CSF)Detected and Quantified21.5 +/- 4.8 uMChildren (1-13 years old)Not SpecifiedLeukemia details
    Cerebrospinal Fluid (CSF)Detected and Quantified 0.00000964 +/- 0.000001244 uMAdult (>18 years old)BothSchizophrenia details
    Cerebrospinal Fluid (CSF)Detected and Quantified16.6 +/- 6.3 uMChildren (1-13 years old)Not Specified
    Leukemia
    details
    Cerebrospinal Fluid (CSF)Detected and Quantified6.0 +/- 1.4 uMAdult (>18 years old)BothAlzheimer's disease details
    Cerebrospinal Fluid (CSF)Detected and Quantified58.78 (0.0-117.56) uMAdult (>18 years old)BothHyperargininemia details
    Cerebrospinal Fluid (CSF)Detected and Quantified78 uMChildren (1-13 years old)MaleArgininemia 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 Children (1-13 years old)BothAutism 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)FemaleMyalgic encephalomyelitis/chronic fatigue syndrome 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
    Colorectal cancer
    details
    SalivaDetected and Quantified15.58 +/- 5.74 uMAdult (>18 years old)MaleAlzheimer's disease details
    SalivaDetected and Quantified18.89 +/- 1.07 uMAdult (>18 years old)MaleFrontotemporal lobe dementia details
    SalivaDetected and Quantified14.27 +/- 1.60 uMAdult (>18 years old)BothLewy body disease details
    UrineDetected and Quantified120-221 umol/mmol creatinineChildren (1-13 years old)BothHyperlysinemia I, familial details
    UrineDetected and Quantified5.400-988.300 umol/mmol creatinineAdult (>18 years old)Both
    Cystinuria
    details
    UrineDetected and Quantified6.6 (2.5-10.8) umol/mmol creatinineAdult (>18 years old)BothCystinuria
      • MetaGene: Metabol...
    details
    UrineDetected and Quantified500.0 (200.0-800.0) umol/mmol creatinineChildren (1-13 years old)BothCystinuria
      • MetaGene: Metabol...
    details
    UrineDetected and Quantified2-153 umol/mmol creatinineChildren (1 - 13 years old)BothLysinuric protein intolerance details
    UrineDetected and Quantified3.635-9.216 umol/mmol creatinineAdult (>18 years old)BothHyperdibasic aminoaciduria I details
    UrineDetected and Quantified488.6 umol/mmol creatinineAdult (>18 years old)Both
    Cystinuria
    details
    UrineDetected and Quantified6.490-10.644 umol/mmol creatinineChildren (1 - 13 years old)FemaleHyperdibasic aminoaciduria I details
    UrineDetected and Quantified35.102 +/- 30.706 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified1941.3 +/- 122.5 umol/mmol creatinineAdult (>18 years old)BothArgininosuccinic aciduria (ASL) details
    UrineDetected and Quantified39.3 umol/mmol creatinineAdult (>18 years old)BothPropionic acidemia details
    UrineDetected and Quantified33.8 +/- 3.2 umol/mmol creatinineAdult (>18 years old)BothTyrosinemia I details
    UrineDetected and Quantified50.3 +/- 10.6 umol/mmol creatinineAdult (>18 years old)BothMaple syrup urine disease details
    UrineDetected and Quantified31.5 +/- 6.7 umol/mmol creatinineAdult (>18 years old)BothAminoaciduria details
    UrineDetected and Quantified2.985-10.644 umol/mmol creatinineChildren (1-13 years old)FemaleDibasic Amino Aciduria I details
    UrineDetected but not Quantified Adult (>18 years old)BothColorectal cancer details
    UrineDetected and Quantified3.635-11.747 umol/mmol creatinineAdult (>18 years old)BothDibasic Amino Aciduria I details
    UrineDetected and Quantified2.0 +/- 0.21 umol/mmol creatinineAdult (>18 years old)BothAlzheimer's disease details
    UrineDetected and Quantified13.2327 +/- 7.0061 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified9.8466 +/- 3.7571 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Gastroesophageal reflux disease
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified54-8426 umol/mmol creatinineAdult (>18 years old)BothHyperlysinuria 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 ]
    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 ]
    Argininemia
    1. Brockstedt M, Smit LM, de Grauw AJ, van der Klei-van Moorsel JM, Jakobs C: A new case of hyperargininaemia: neurological and biochemical findings prior to and during dietary treatment. Eur J Pediatr. 1990 Feb;149(5):341-3. [PubMed:2311630 ]
    2. Schlune A, Vom Dahl S, Haussinger D, Ensenauer R, Mayatepek E: Hyperargininemia due to arginase I deficiency: the original patients and their natural history, and a review of the literature. Amino Acids. 2015 Sep;47(9):1751-62. doi: 10.1007/s00726-015-2032-z. Epub 2015 Jun 27. [PubMed:26123990 ]
    3. Michels VV, Beaudet AL: Arginase deficiency in multiple tissues in argininemia. Clin Genet. 1978 Jan;13(1):61-7. [PubMed:624188 ]
    4. Therrell BL, Currier R, Lapidus D, Grimm M, Cederbaum SD: Newborn screening for hyperargininemia due to arginase 1 deficiency. Mol Genet Metab. 2017 Aug;121(4):308-313. doi: 10.1016/j.ymgme.2017.06.003. Epub 2017 Jun 20. [PubMed:28659245 ]
    5. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
    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 ]
    Chronic renal failure
    1. Kikuchi T, Orita Y, Ando A, Mikami H, Fujii M, Okada A, Abe H: Liquid-chromatographic determination of guanidino compounds in plasma and erythrocyte of normal persons and uremic patients. Clin Chem. 1981 Nov;27(11):1899-902. [PubMed:7296840 ]
    Citrullinemia type I
    1. Kose E, Unal O, Bulbul S, Gunduz M, Haberle J, Arslan N: Identification of three novel mutations in fourteen patients with citrullinemia type 1. Clin Biochem. 2017 Aug;50(12):686-689. doi: 10.1016/j.clinbiochem.2017.01.011. Epub 2017 Jan 27. [PubMed:28132756 ]
    Colorectal cancer
    1. 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 ]
    2. 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 ]
    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. 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 ]
    Crohn's disease
    1. 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 ]
    Cystinuria
    1. Fjellstedt E, Harnevik L, Jeppsson JO, Tiselius HG, Soderkvist P, Denneberg T: Urinary excretion of total cystine and the dibasic amino acids arginine, lysine and ornithine in relation to genetic findings in patients with cystinuria treated with sulfhydryl compounds. Urol Res. 2003 Dec;31(6):417-25. Epub 2003 Oct 25. [PubMed:14586528 ]
    2. 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. He Y, Yu Z, Giegling I, Xie L, Hartmann AM, Prehn C, Adamski J, Kahn R, Li Y, Illig T, Wang-Sattler R, Rujescu D: Schizophrenia shows a unique metabolomics signature in plasma. Transl Psychiatry. 2012 Aug 14;2:e149. doi: 10.1038/tp.2012.76. [PubMed:22892715 ]
    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 ]
    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 ]
    Hyperargininemia
    1. Mizutani N, Hayakawa C, Ohya Y, Watanabe K, Watanabe Y, Mori A: Guanidino compounds in hyperargininemia. Tohoku J Exp Med. 1987 Nov;153(3):197-205. [PubMed:3433275 ]
    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 ]
    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 ]
    Lysinuric protein intolerance
    1. Habib A, Azize NA, Yakob Y, Md Yunus Z, Wee TK: Biochemical and molecular characteristics of Malaysian patients with lysinuric protein intolerance. Malays J Pathol. 2016 Dec;38(3):305-310. [PubMed:28028301 ]
    2. Kurko J, Tringham M, Tanner L, Nanto-Salonen K, Vaha-Makila M, Nygren H, Poho P, Lietzen N, Mattila I, Olkku A, Hyotylainen T, Oresic M, Simell O, Niinikoski H, Mykkanen J: Imbalance of plasma amino acids, metabolites and lipids in patients with lysinuric protein intolerance (LPI). Metabolism. 2016 Sep;65(9):1361-75. doi: 10.1016/j.metabol.2016.05.012. Epub 2016 May 27. [PubMed:27506743 ]
    3. Olli Simell (1995). Lysinuric Protein Intolerance and Other Cationic Aminoacidurias. The metabolic and molecular bases of inherited disease, 7/e; Editors: C.R.Scriver, A.L.Beaudet, W.S.Sly, D.Valle; McGraw-Hill Inc. (1995) DOI: 10.1036/ommbid.225. McGraw-Hill Inc..
    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.
    Intestinal failure
    1. Crenn P, Coudray-Lucas C, Thuillier F, Cynober L, Messing B: Postabsorptive plasma citrulline concentration is a marker of absorptive enterocyte mass and intestinal failure in humans. Gastroenterology. 2000 Dec;119(6):1496-505. [PubMed:11113071 ]
    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 ]
    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 ]
    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 ]
    2. 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 ]
    Hyperdibasic aminoaciduria I
    1. Whelan DT, Scriver CR: Hyperdibasicaminoaciduria: an inherited disorder of amino acid transport. Pediatr Res. 1968 Nov;2(6):525-34. [PubMed:5727921 ]
    Eosinophilic esophagitis
    1. (). Mordechai, Hien, and David S. Wishart. .
    Citrullinemia type II, neonatal-onset
    1. Ohura T, Kobayashi K, Tazawa Y, Nishi I, Abukawa D, Sakamoto O, Iinuma K, Saheki T: Neonatal presentation of adult-onset type II citrullinemia. Hum Genet. 2001 Feb;108(2):87-90. [PubMed:11281457 ]
    Cutis laxa, autosomal recessive, type IIIA
    1. Baumgartner MR, Hu CA, Almashanu S, Steel G, Obie C, Aral B, Rabier D, Kamoun P, Saudubray JM, Valle D: Hyperammonemia with reduced ornithine, citrulline, arginine and proline: a new inborn error caused by a mutation in the gene encoding delta(1)-pyrroline-5-carboxylate synthase. Hum Mol Genet. 2000 Nov 22;9(19):2853-8. [PubMed:11092761 ]
    Duchenne Muscular Dystrophy
    1. Horster I, Weigt-Usinger K, Carmann C, Chobanyan-Jurgens K, Kohler C, Schara U, Kayacelebi AA, Beckmann B, Tsikas D, Lucke T: The L-arginine/NO pathway and homoarginine are altered in Duchenne muscular dystrophy and improved by glucocorticoids. Amino Acids. 2015 Sep;47(9):1853-63. doi: 10.1007/s00726-015-2018-x. Epub 2015 Jun 12. [PubMed:26066683 ]
    Hyperlysinemia I, familial
    1. Tondo M, Calpena E, Arriola G, Sanz P, Martorell L, Ormazabal A, Castejon E, Palacin M, Ugarte M, Espinos C, Perez B, Perez-Duenas B, Perez-Cerda C, Artuch R: Clinical, biochemical, molecular and therapeutic aspects of 2 new cases of 2-aminoadipic semialdehyde synthase deficiency. Mol Genet Metab. 2013 Nov;110(3):231-6. doi: 10.1016/j.ymgme.2013.06.021. Epub 2013 Jul 6. [PubMed:23890588 ]
    Hyperlysinuria
    1. Fjellstedt E, Harnevik L, Jeppsson JO, Tiselius HG, Soderkvist P, Denneberg T: Urinary excretion of total cystine and the dibasic amino acids arginine, lysine and ornithine in relation to genetic findings in patients with cystinuria treated with sulfhydryl compounds. Urol Res. 2003 Dec;31(6):417-25. Epub 2003 Oct 25. [PubMed:14586528 ]
    Myopathy, lactic acidosis, and sideroblastic anemia 1
    1. Parfait B, de Lonlay P, von Kleist-Retzow JC, Cormier-Daire V, Chretien D, Rotig A, Rabier D, Saudubray JM, Rustin P, Munnich A: The neurogenic weakness, ataxia and retinitis pigmentosa (NARP) syndrome mtDNA mutation (T8993G) triggers muscle ATPase deficiency and hypocitrullinaemia. Eur J Pediatr. 1999 Jan;158(1):55-8. [PubMed:9950309 ]
    Pearson Syndrome
    1. Crippa BL, Leon E, Calhoun A, Lowichik A, Pasquali M, Longo N: Biochemical abnormalities in Pearson syndrome. Am J Med Genet A. 2015 Mar;167A(3):621-8. doi: 10.1002/ajmg.a.36939. [PubMed:25691415 ]
    Phosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic
    1. Vieira P, Cameron J, Rahikkala E, Keski-Filppula R, Zhang LH, Santra S, Matthews A, Myllynen P, Nuutinen M, Moilanen JS, Rodenburg RJ, Rolfs A, Uusimaa J, van Karnebeek CDM: Novel homozygous PCK1 mutation causing cytosolic phosphoenolpyruvate carboxykinase deficiency presenting as childhood hypoglycemia, an abnormal pattern of urine metabolites and liver dysfunction. Mol Genet Metab. 2017 Apr;120(4):337-341. doi: 10.1016/j.ymgme.2017.02.003. Epub 2017 Feb 6. [PubMed:28216384 ]
    2. Santra S, Cameron JM, Shyr C, Zhang L, Drogemoller B, Ross CJ, Wasserman WW, Wevers RA, Rodenburg RJ, Gupte G, Preece MA, van Karnebeek CD: Cytosolic phosphoenolpyruvate carboxykinase deficiency presenting with acute liver failure following gastroenteritis. Mol Genet Metab. 2016 May;118(1):21-7. doi: 10.1016/j.ymgme.2016.03.001. Epub 2016 Mar 4. [PubMed:26971250 ]
    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. 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 ]
    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 IDDB00125
    Phenol Explorer Compound IDNot Available
    FoodDB IDFDB002257
    KNApSAcK IDC00001340
    Chemspider ID6082
    KEGG Compound IDC00062
    BioCyc IDARG
    BiGG ID33707
    Wikipedia LinkL-arginine
    METLIN ID5502
    PubChem Compound6322
    PDB ID1FOL
    ChEBI ID16467
    References
    Synthesis ReferenceMeyer, Helmut E.; Swiderek, Kristine; Hoffmann-Posorske, Edeltraut; Korte, Horst; Heilmeyer, Ludwig M. G., Jr. Quantitative determination of phosphoserine by high-performance liquid chromatography as the phenylthiocarbamyl-S-ethylcysteine. Application to
    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. 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 ]
    3. 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 ]
    4. 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 ]
    5. 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 ]
    6. Mizutani N, Hayakawa C, Ohya Y, Watanabe K, Watanabe Y, Mori A: Guanidino compounds in hyperargininemia. Tohoku J Exp Med. 1987 Nov;153(3):197-205. [PubMed:3433275 ]
    7. Haas W, Grabe K, Geis C, Pach T, Stoll K, Fuchs M, Haberl B, Loy C: Recognition and invasion of human skin by Schistosoma mansoni cercariae: the key-role of L-arginine. Parasitology. 2002 Feb;124(Pt 2):153-67. [PubMed:11860033 ]
    8. Mori A, Watanabe Y, Fujimoto N: Fluorometrical analysis of guanidino compounds in human cerebrospinal fluid. J Neurochem. 1982 Feb;38(2):448-50. [PubMed:7108550 ]
    9. Martens-Lobenhoffer J, Bode-Boger SM: Measurement of asymmetric dimethylarginine (ADMA) in human plasma: from liquid chromatography estimation to liquid chromatography-mass spectrometry quantification. Eur J Clin Pharmacol. 2006 Feb;62(Supplement 13):61-68. [PubMed:16217682 ]
    10. Stothers L, Laher I, Christ GT: A review of the L-arginine - nitric oxide - guanylate cyclase pathway as a mediator of lower urinary tract physiology and symptoms. Can J Urol. 2003 Oct;10(5):1971-80. [PubMed:14633324 ]
    11. Avogaro A, Toffolo G, Kiwanuka E, de Kreutzenberg SV, Tessari P, Cobelli C: L-arginine-nitric oxide kinetics in normal and type 2 diabetic subjects: a stable-labelled 15N arginine approach. Diabetes. 2003 Mar;52(3):795-802. [PubMed:12606522 ]
    12. Kotikoski H, Moilanen E, Vapaatalo H, Aine E: Biochemical markers of the L-arginine-nitric oxide pathway in the aqueous humour in glaucoma patients. Acta Ophthalmol Scand. 2002 Apr;80(2):191-5. [PubMed:11952488 ]
    13. Noris M, Todeschini M, Cassis P, Pasta F, Cappellini A, Bonazzola S, Macconi D, Maucci R, Porrati F, Benigni A, Picciolo C, Remuzzi G: L-arginine depletion in preeclampsia orients nitric oxide synthase toward oxidant species. Hypertension. 2004 Mar;43(3):614-22. Epub 2004 Jan 26. [PubMed:14744923 ]
    14. Kirkeby HJ, Svane D, Poulsen J, Tottrup A, Forman A, Andersson KE: Role of the L-arginine/nitric oxide pathway in relaxation of isolated human penile cavernous tissue and circumflex veins. Acta Physiol Scand. 1993 Nov;149(3):385-92. [PubMed:8310843 ]
    15. Crenn P, Coudray-Lucas C, Thuillier F, Cynober L, Messing B: Postabsorptive plasma citrulline concentration is a marker of absorptive enterocyte mass and intestinal failure in humans. Gastroenterology. 2000 Dec;119(6):1496-505. [PubMed:11113071 ]
    16. Bauchart-Thevret C, Cui L, Wu G, Burrin DG: Arginine-induced stimulation of protein synthesis and survival in IPEC-J2 cells is mediated by mTOR but not nitric oxide. Am J Physiol Endocrinol Metab. 2010 Dec;299(6):E899-909. doi: 10.1152/ajpendo.00068.2010. Epub 2010 Sep 14. [PubMed:20841502 ]
    17. Linden KC, Wadley GD, Garnham AP, McConell GK: Effect of l-arginine infusion on glucose disposal during exercise in humans. Med Sci Sports Exerc. 2011 Sep;43(9):1626-34. doi: 10.1249/MSS.0b013e318212a317. [PubMed:21311355 ]

    Only showing the first 10 proteins. There are 25 proteins in total.

    Enzymes

    General function:
    Involved in arginase activity
    Specific function:
    Not Available
    Gene Name:
    ARG1
    Uniprot ID:
    P05089
    Molecular weight:
    34734.655
    Reactions
    L-Arginine + Water → Ornithine + Ureadetails
    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
    L-Arginine + gamma-Aminobutyric acid → Ornithine + 4-Guanidinobutanoic aciddetails
    General function:
    Involved in nucleotide binding
    Specific function:
    Forms part of a macromolecular complex that catalyzes the attachment of specific amino acids to cognate tRNAs during protein synthesis. Modulates the secretion of AIMP1 and may be involved in generation of the inflammatory cytokine EMAP2 from AIMP1.
    Gene Name:
    RARS
    Uniprot ID:
    P54136
    Molecular weight:
    75378.295
    Reactions
    Adenosine triphosphate + L-Arginine + tRNA(Arg) → Adenosine monophosphate + Pyrophosphate + L-arginyl-tRNA(Arg)details
    Adenosine triphosphate + L-Arginine + tRNA(Arg) → Adenosine monophosphate + Pyrophosphate + L-Arginyl-tRNA(Arg)details
    General function:
    Involved in argininosuccinate synthase activity
    Specific function:
    Not Available
    Gene Name:
    ASS1
    Uniprot ID:
    P00966
    Molecular weight:
    46530.055
    General function:
    Involved in protein-arginine deiminase activity
    Specific function:
    Catalyzes the citrullination/deimination of arginine residues of proteins. Citrullinates histone H3 at 'Arg-8' and/or 'Arg-17' and histone H4 at 'Arg-3', which prevents their methylation by CARM1 and HRMT1L2/PRMT1 and represses transcription. Citrullinates EP300/P300 at 'Arg-2142', which favors its interaction with NCOA2/GRIP1.
    Gene Name:
    PADI4
    Uniprot ID:
    Q9UM07
    Molecular weight:
    74078.65
    General function:
    Involved in protein-arginine deiminase activity
    Specific function:
    Catalyzes the deimination of arginine residues of proteins.
    Gene Name:
    PADI3
    Uniprot ID:
    Q9ULW8
    Molecular weight:
    74742.705
    General function:
    Involved in argininosuccinate lyase activity
    Specific function:
    Not Available
    Gene Name:
    ASL
    Uniprot ID:
    P04424
    Molecular weight:
    51657.505
    Reactions
    Argininosuccinic acid → Fumaric acid + L-Argininedetails
    General function:
    Involved in zinc ion binding
    Specific function:
    NAD-dependent protein deacetylase. Has deacetylase activity towards histone H3K9Ac and H3K56Ac. Modulates acetylation of histone H3 in telomeric chromatin during the S-phase of the cell cycle. Deacetylates histone H3K9Ac at NF-kappa-B target promoters and may down-regulate the expression of a subset of NF-kappa-B target genes. Acts as a corepressor of the transcription factor HIF1A to control the expression of multiple glycolytic genes to regulate glucose homeostasis. Required for genomic stability. Regulates the production of TNF protein. Has a role in the regulation of life span (By similarity). Deacetylation of nucleosomes interferes with RELA binding to target DNA. May be required for the association of WRN with telomeres during S-phase and for normal telomere maintenance. Required for genomic stability. Required for normal IGF1 serum levels and normal glucose homeostasis. Modulates cellular senescence and apoptosis. On DNA damage, promotes DNA end resection via deacetylation of RBBP8. Has very weak deacetylase activity and can bind NAD(+) in the absence of acetylated substrate.
    Gene Name:
    SIRT6
    Uniprot ID:
    Q8N6T7
    Molecular weight:
    36064.295
    General function:
    Involved in NAD(P)+-protein-arginine ADP-ribosyltransferase activity
    Specific function:
    Not Available
    Gene Name:
    ART3
    Uniprot ID:
    Q13508
    Molecular weight:
    43922.97
    General function:
    Involved in NAD(P)+-protein-arginine ADP-ribosyltransferase activity
    Specific function:
    Not Available
    Gene Name:
    ART5
    Uniprot ID:
    Q96L15
    Molecular weight:
    32053.48

    Only showing the first 10 proteins. There are 25 proteins in total.