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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-05-20 20:40:48 UTC
HMDB IDHMDB0000214
Secondary Accession Numbers
  • HMDB0000818
  • HMDB0004984
  • HMDB00214
  • HMDB00818
  • HMDB04984
Metabolite Identification
Common NameOrnithine
DescriptionL-ornithine, also known as (S)-2,5-diaminopentanoic acid or ornithine is a member of the class of compounds known as L-alpha-amino acids. L-alpha-amino acids are alpha amino acids which have the L-configuration of the alpha-carbon atom. L-ornithine is soluble (in water) and a moderately acidic compound. It has been claimed that ornithine improves athletic performance, has anabolic effects, has wound-healing effects, and is immuno-enhancing. L-ornithine is abundant in a number of food items such as wild rices, brazil nuts, common oregano, and common grapes. L-ornithine can be found throughout most human tissues; and in most biofluids, some of which include blood, urine, cerebrospinal fluid (CSF), sweat, saliva, and feces. Within the cell, L-ornithine is located in the mitochondria and the cytoplasm. L-ornithine exists in all living species, from bacteria to humans. In humans, L-ornithine is involved in numerous metabolic disorders, some of which include, ornithine transcarbamylase deficiency (OTC deficiency), argininemia, and guanidinoacetate methyltransferase deficiency (GAMT deficiency). Moreover, Ornithine is found to be associated with cystinuria, hyperdibasic aminoaciduria I, and lysinuric protein intolerance, which are inborn errors of metabolism. Ornithine is produced in the urea cycle through the cleavage of urea from arginine. It is a central part of the urea cycle, which allows for the disposal of excess nitrogen. L-Ornithine is also a precursor of citrulline and arginine. In order for ornithine produced in the cytosol to be converted to citrulline, it must first cross the inner mitochondrial membrane into the mitochondrial matrix where it is carbamylated by ornithine transcarbamylase. This transfer is mediated by the mitochondrial ornithine transporter (SLC25A15; AF112968; ORNT1). Mutations in the mitochondrial ornithine transporter result in hyperammonemia, hyperornithinemia, homocitrullinuria (HHH) syndrome, a disorder of the urea cycle (PMID: 16256388 ). The pathophysiology of the disease may involve diminished ornithine transport into mitochondria, resulting in ornithine accumulation in the cytoplasm and reduced ability to clear carbamoyl phosphate and ammonia loads (OMIM 838970 ).
Structure
Thumb
Synonyms
ValueSource
(S)-2,5-DiaminopentanoateChEBI
(S)-2,5-Diaminopentanoic acidChEBI
(S)-2,5-Diaminovaleric acidChEBI
(S)-alpha,delta-Diaminovaleric acidChEBI
(S)-OrnithineChEBI
(S)-2,5-DiaminovalerateGenerator
(S)-a,delta-DiaminovalerateGenerator
(S)-a,delta-Diaminovaleric acidGenerator
(S)-alpha,delta-DiaminovalerateGenerator
(S)-α,δ-diaminovalerateGenerator
(S)-α,δ-diaminovaleric acidGenerator
(S)-a,δ-diaminovalerateGenerator
(S)-a,δ-diaminovaleric acidGenerator
(+)-S-OrnithineHMDB
(S)-a,D-DiaminovalerateHMDB
(S)-a,D-Diaminovaleric acidHMDB
5-amino-L-NorvalineHMDB
L-(-)-OrnithineHMDB
L-OrnithineHMDB
2,5-Diaminopentanoic acidMeSH
Ornithine dihydrochloride, (L)-isomerMeSH
Ornithine hydrochloride, (D)-isomerMeSH
Ornithine, (D)-isomerMeSH
Ornithine, (L)-isomerMeSH
2,5 Diaminopentanoic acidMeSH
Ornithine monoacetate, (L)-isomerMeSH
Ornithine monohydrochloride, (D)-isomerMeSH
Ornithine monohydrochloride, (DL)-isomerMeSH
Ornithine phosphate (1:1), (L)-isomerMeSH
Ornithine sulfate (1:1), (L)-isomerMeSH
Ornithine hydrochloride, (DL)-isomerMeSH
Ornithine hydrochloride, (L)-isomerMeSH
Ornithine monohydrobromide, (L)-isomerMeSH
Ornithine, (DL)-isomerMeSH
Chemical FormulaC5H12N2O2
Average Molecular Weight132.161
Monoisotopic Molecular Weight132.089877638
IUPAC Name(2S)-2,5-diaminopentanoic acid
Traditional Nameornithine
CAS Registry Number3184-13-2
SMILES
NCCC[C@H](N)C(O)=O
InChI Identifier
InChI=1S/C5H12N2O2/c6-3-1-2-4(7)5(8)9/h4H,1-3,6-7H2,(H,8,9)/t4-/m0/s1
InChI KeyAHLPHDHHMVZTML-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
  • Amino acid
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Amine
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Organic oxygen compound
  • Primary aliphatic amine
  • 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:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point140 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility620 mg/mLNot Available
LogP-4.22SANGSTER (1994)
Predicted Properties
PropertyValueSource
Water Solubility172 g/LALOGPS
logP-3.6ALOGPS
logP-3.7ChemAxon
logS0.11ALOGPS
pKa (Strongest Acidic)2.67ChemAxon
pKa (Strongest Basic)10.29ChemAxon
Physiological Charge1ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area89.34 ŲChemAxon
Rotatable Bond Count4ChemAxon
Refractivity33.21 m³·mol⁻¹ChemAxon
Polarizability13.85 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) (2 TMS)splash10-0006-1920000000-b0357b8c9888abda7d80View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0006-0910000000-8aaa0faabf1429b90611View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-00di-7910000000-5fa118444ceb45204aa3View in MoNA
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-006x-3910000000-f6d0c6492f2cab33a392View in MoNA
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-00dr-2900000000-77222ff2d0118d3d3b57View in MoNA
GC-MSGC-MS Spectrum - GC-MS (4 TMS)splash10-0006-1910000000-53c73c4fa3665c44d3b9View in MoNA
GC-MSGC-MS Spectrum - GC-MS (5 TMS)splash10-03di-1691000000-dc14976cfee86cc828b9View in MoNA
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0006-0920000000-f65f61e4a3739eabb1d1View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0006-1920000000-b0357b8c9888abda7d80View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0006-0910000000-8aaa0faabf1429b90611View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00di-7910000000-5fa118444ceb45204aa3View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-006x-3910000000-f6d0c6492f2cab33a392View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00dr-2900000000-77222ff2d0118d3d3b57View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0006-1910000000-53c73c4fa3665c44d3b9View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-03di-1691000000-dc14976cfee86cc828b9View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0006-0900000000-f59681b9f46f1b738487View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0006-0900000000-3048477975b51ae176afView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00dr-2900000000-8d1a2cc0a88f7d9216e1View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0udi-1900000000-bc40b76e8b03ef7a08f0View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-001l-9000000000-50fe14c2cb68fd09e32fView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-0089-9300000000-bbfdea03043e52ad8a15View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-01b9-6900000000-8fe592b77d2d8e4ae704View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00di-9000000000-462b84db532c33527580View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-00di-9000000000-9c9c28d2e40d5eadacd2View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-e44e06092c06bb4791afView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-014i-9000000000-4d6ccc429f5155b24654View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-014i-0900000000-dcad699e8ed7e1b19860View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-7b130437d4e1ceea277bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-0fae230bc6b21623a4bfView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-014i-9000000000-5a350a8e7c607a8b9a60View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-014i-0900000000-516054b6c2f3cd4a1d02View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-000i-0793321000-9fef988b5ce019f84642View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-03di-0900000000-a612df8df5f8ba1ff7f3View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-001i-0910000000-97fad20414656e2b7b93View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-01di-0490000000-1b137b937beb110d2e4bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-001i-0900000000-e3fc95eb909e45d8e648View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-001i-1900000000-cdd9aac89a5a71814ff3View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-001r-9500000000-f1cc3eccf3f5ab734222View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-0159-0900000000-93ce7b142d1f82f17e95View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-00di-9200000000-186f270471bdec40f804View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-00di-9000000000-ebbdf0ab6d3180cba1a5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-00di-9000000000-207bcc826fb2238c2cc5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-00di-9000000000-a2f9a950fb23a6b18857View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-01c9-9700000000-0364d35bb1a7c10a09c7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00dr-9100000000-9c4015733140885b4957View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-00dl-9000000000-b32e7180e30a835008bbView in MoNA
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
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Sweat
  • Urine
Tissue Location
  • Gut
  • Liver
  • Prostate
  • Skin
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified55.0 (39.0-71.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified93.8 +/- 41.3 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified27-86 uMAdult (>18 years old)Not SpecifiedNormal
    • Lysinuric Protein...
details
BloodDetected and Quantified90.0 +/- 20.0 uMNewborn (0-30 days old)Not SpecifiedNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified47.0 +/- 14.0 uMChildren (1-13 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified65.0 +/- 18.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified54.0 +/- 18.0 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified109.0 +/- 32.0 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified34.3 +/- 13.0 uMChildren (1-13 years old)BothNormal details
BloodDetected and Quantified185 (144-222) uMNewborn (0-30 days old)Not Available
Normal
details
BloodDetected and Quantified31-179 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified124 (90.4-189) uMInfant (0-1 year old)Not Available
Normal
details
BloodDetected but not Quantified Adult (>18 years old)Both
Normal
details
BloodDetected and Quantified30-64 uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified72 +/- 25 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified20-135 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified20-135 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified54 +/- 18 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified30.00-110.0 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified69.0 +/- 18.0 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified65 +/- 18 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified69.1(56.1-90.2) uMChildren (1-13 uears old)Both
Normal
details
BloodDetected and Quantified10-110 uMChildren (1-13 years old)BothNormal details
BloodDetected and Quantified48-211 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified74.3 (52.7-81.3) uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified77 +/- 41 uMInfant (0-1 year old)BothNormal details
BloodDetected and Quantified8-156 uMChildren (1 - 13 years old)BothNormal details
BloodDetected and Quantified38-207 uMNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified26-117 uMInfant (1 - 3 months old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified27-100 uMChildren (3 months - 6 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified19-100 uMChildren (6 - 18 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified66.9 +/- 15.3 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified4.87 (3.48-6.26) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified8.3 +/- 4.7 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified6.0 +/- 1.5 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified4.9 +/- 1.6 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified3.7 +/- 1.0 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified8.1(5-7-10-9) uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified2.4-9.0 uMChildren (1-13 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified4.5 +/- 2.2 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified2.3-9.5 uMChildren (0 - 10 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified2.3-11.2 uMAdolescent (>11 years old)Both
Normal
    • BC Children's Hos...
details
FecesDetected but not Quantified Infant (0-1 year old)Both
Normal
details
FecesDetected but not Quantified Children (1-13 years old)BothNormal details
FecesDetected but not Quantified Not SpecifiedBoth
Normal
details
FecesDetected but not Quantified 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 Children (6 - 18 years old)BothNormal details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedNormal details
FecesDetected and Quantified20 +/- 20 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Not SpecifiedNot Specified
Normal
details
SalivaDetected and Quantified12.5 +/- 10.8 uMAdult (>18 years old)Not Specified
Normal
    • Physiological and...
details
SalivaDetected and Quantified14.2 +/- 11.1 uMAdult (>18 years old)Female
Normal
    • Physiological and...
details
SalivaDetected and Quantified16.7 +/- 14.6 uMAdult (>18 years old)Not Specified
Normal
    • Physiological and...
details
SalivaDetected and Quantified18.3 +/- 14.2 uMAdult (>18 years old)Not Specified
Normal
    • Physiological and...
details
SalivaDetected and Quantified28.9 +/- 33.1 uMAdult (>18 years old)Female
Normal
    • Physiological and...
details
SalivaDetected and Quantified28.9 +/- 33.1 uMAdult (>18 years old)Not Specified
Normal
    • Physiological and...
details
SalivaDetected but not Quantified Adult (>18 years old)BothNormal details
SalivaDetected and Quantified13.4 +/- 7.5 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified11.5 +/- 6.3 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified14.0 +/- 10.6 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified65.34 +/- 15.64 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected and Quantified18.5 +/- 8.6 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified>10 uMAdult (>18 years old)BothNormal details
SalivaDetected and Quantified38.16 +/- 52.83 uMAdult (>18 years old)FemaleNormal details
SweatDetected and Quantified< 10 uMAdult (60 years old)Male
Normal
details
SweatDetected and Quantified< 10 uMAdult (40 years old)Male
Normal
details
SweatDetected but not Quantified Adult BothNormal details
UrineDetected and Quantified0-5 umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified5.3 (2.0-8.8) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.98 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected but not Quantified Adult (>18 years old)BothNormal details
UrineDetected and Quantified12.584 +/- 12.605 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified0 - 24.08 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified2.19 +/- 3.21 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified0 - 13.00 umol/mmol creatinineInfant (1 - 6 months old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 10.40 umol/mmol creatinineInfant (6 months - <1 year old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 10.40 umol/mmol creatinineChildren (1 - 2 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 9.04 umol/mmol creatinineChildren (2 - 4 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 9.04 umol/mmol creatinineChildren (4 - 13 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 6.56 umol/mmol creatinineAdolescent (13 - 21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 6.56 umol/mmol creatinineAdult (>21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified1.5-2.6 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified2.733 +/- 1.313 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified2.0 (0.90-2.94) umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified3.147 +/- 1.487 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
UrineDetected but not Quantified Adult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.0024 - 0.0111 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.787 +/- 0.373 umol/mmol creatinineChildren (1 - 13 years old)Not SpecifiedNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified0.593 +/- 0.340 umol/mmol creatinineChildren (1 - 13 years old)Not SpecifiedNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified0.987 (0.132-1.842) umol/mmol creatinineAdult (>18 years old)BothNormal
    details
    UrineDetected and Quantified1.882 +/- 1.968 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
    UrineDetected and Quantified4.11 +/- 1.37 umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected and Quantified4.4 (1.2-22.1) umol/mmol creatinineAdult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified0.050 (0.0-0.098) umol/mmol creatinineNewborn (0-30 days old)BothNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified0.78 +/- 0.36 umol/mmol creatinineChildren (1-13 years old)MaleNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified3.1 +/- 1.46 umol/mmol creatinineAdult (>18 years old)MaleNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified2.6 +/- 1.30 umol/mmol creatinineAdult (>18 years old)FemaleNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified0-7 umol/mmol creatinineChildren (1 - 13 years old)BothNormal details
    UrineDetected and Quantified1.696-46.468 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedNormal details
    UrineDetected and Quantified1.882 +/- 1.968 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
    UrineDetected and Quantified1.2-2.3 umol/mmol creatinineAdult (>18 years old)MaleNormal details
    UrineDetected and Quantified0.9 umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected and Quantified2.471 +/- 0.8319 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Normal
      • Mordechai, Hien, ...
    details
    Abnormal Concentrations
    BiospecimenStatusValueAgeSexConditionReferenceDetails
    BloodDetected and Quantified30.5 (12.1) uMAdult (>18 years old)FemaleDown syndrome pregnancy details
    BloodDetected and Quantified55.2 +/- 15.4 uMChildren (1-13 years old)Both
    Obesity
      • Metabolomics reve...
    details
    BloodDetected and Quantified26.0 (10.8) uMAdult (>18 years old)FemalePregnancy details
    BloodDetected and Quantified60.2 +/- 18.2 uMChildren (1-13 years old)Both
    Obesity
      • Metabolomics reve...
    details
    BloodDetected and Quantified65.4 +/- 30.4 uMAdult (>18 years old)BothHeart Transplant details
    BloodDetected and Quantified21 (2-83) uMAdult (>18 years old)Not SpecifiedLysinuric protein intolerance
      • Lysinuric Protein...
    details
    BloodDetected and Quantified41.0 +/- 16.0 uMAdult (>18 years old)BothOrnithine transcarbamylase (OTC) deficiency details
    BloodDetected and Quantified36.83 +/- 13.32 uMAdult (>18 years old)Female
    Pregnancy with fetuses with trisomy 18
    details
    BloodDetected and Quantified28.20 +/- 10.61 uMAdult (>18 years old)FemalePregnancy details
    BloodDetected and Quantified37.8 +/- 12.0 uMChildren (1-13 years old)BothUremia details
    BloodDetected and Quantified36.1 (11.9) uMAdult (>18 years old)FemaleEarly preeclampsia details
    BloodDetected and Quantified38.2 (15.7) uMAdult (>18 years old)FemalePregnancy details
    BloodDetected but not Quantified Adult (>18 years old)BothColorectal cancer details
    BloodDetected and Quantified191 uMNewborn (0-30 days old)Male
    Narp Syndrome
    details
    BloodDetected and Quantified23-54 uMChildren (1-13 years old)MaleNarp Syndrome details
    BloodDetected and Quantified20-153 uMInfant (0-1 year old)BothNarp Syndrome details
    BloodDetected but not Quantified Adult (>18 years old)Both
    Schizophrenia
    details
    BloodDetected and Quantified25.15 (7.57) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
    BloodDetected and Quantified22.37 (8.47) uMAdult (>18 years old)FemalePregnancy details
    BloodDetected and Quantified33 +/- 29 (9-98) uMChildren (1-13 years old)Female
    Pearson Syndrome
    details
    BloodDetected and Quantified1066(798-1321) uMAdult (>18 years old)BothHyperornithinemia with gyrate atrophy details
    BloodDetected and Quantified33 +/- 15 uMChildren (1-13 years old)BothCutis laxa, autosomal recessive, type IIIA details
    BloodDetected and Quantified19 uMInfant (0-1 year old)FemalePearson Syndrome details
    BloodDetected and Quantified36.8 (17.4) uMAdult (>18 years old)FemaleLate-onset preeclampsia details
    BloodDetected and Quantified42.3 (22.5) uMAdult (>18 years old)FemalePregnancy details
    BloodDetected and Quantified74.9(54.3-116.5) uMChildren (1-13 uears old)Both
    Environmental enteric dysfunction
    details
    BloodDetected and Quantified68.35 +/- 22.43 uMAdult (>18 years old)BothAlzheimer's disease details
    BloodDetected and Quantified77 uMInfant (0-1 year old)MaleFumaric aciduria details
    BloodDetected and Quantified32-167 uMNewborn (0-30 days old)MaleN-Acetylglutamate synthetase deficiency details
    BloodDetected and Quantified32.3 (22.4-53.9) uMAdult (>18 years old)Both
    Lysinuric protein intolerance
    details
    BloodDetected and Quantified4-22 uMChildren (1 - 13 years old)BothLysinuric protein intolerance details
    BloodDetected and Quantified42-290 uMChildren (1-13 years old)Male
    N-acetylglutamate synthetase deficiency
    details
    BloodDetected and Quantified53-152 uMInfant (0-1 year old)Male
    N-Acetylglutamate synthetase deficiency
    details
    Cerebrospinal Fluid (CSF)Detected and Quantified7.6 +/- 3.1 uMChildren (1-13 years old)Not SpecifiedLeukemia details
    Cerebrospinal Fluid (CSF)Detected and Quantified6.9 +/- 3.6 uMChildren (1-13 years old)Not Specified
    Leukemia
    details
    Cerebrospinal Fluid (CSF)Detected and Quantified288(240-314) uMAdult (>18 years old)BothHyperornithinemia with gyrate atrophy details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Colorectal cancer
    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
    Colorectal cancer
    details
    SalivaDetected but not Quantified Adult (>18 years old)BothOral cancer details
    SalivaDetected but not Quantified Adult (>18 years old)FemaleBreast cancer details
    SalivaDetected but not Quantified Adult (>18 years old)Not SpecifiedPancreatic cancer details
    SalivaDetected but not Quantified Adult (>18 years old)Not SpecifiedPeriodontal diseases details
    SalivaDetected and Quantified28.26 +/- 9.78 uMAdult (>18 years old)MaleAlzheimer's disease details
    SalivaDetected and Quantified24.28 +/- 12.91 uMAdult (>18 years old)MaleFrontotemporal lobe dementia details
    SalivaDetected and Quantified16.85 +/- 10.62 uMAdult (>18 years old)BothLewy body disease details
    UrineDetected and Quantified26-63 umol/mmol creatinineChildren (1-13 years old)BothHyperlysinemia I, familial details
    UrineDetected and Quantified19-988.300 umol/mmol creatinineAdult (>18 years old)Both
    Cystinuria
    details
    UrineDetected and Quantified11.611 +/- 17.647 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified5.0 (2.0-8.0) umol/mmol creatinineAdult (>18 years old)BothCystinuria
      • MetaGene: Metabol...
    details
    UrineDetected and Quantified350.0 (200.0-500.0) umol/mmol creatinineChildren (1-13 years old)BothCystinuria
      • MetaGene: Metabol...
    details
    UrineDetected and Quantified4.363-53.381 umol/mmol creatinineChildren (1-13 years old)FemaleDibasic Amino Aciduria I details
    UrineDetected and Quantified4.106-35.759 umol/mmol creatinineAdult (>18 years old)BothDibasic Amino Aciduria I details
    UrineDetected and Quantified12.34 +/- 5.07 umol/mmol creatinineAdult (>18 years old)BothAlzheimer's disease details
    UrineDetected and Quantified190-9883 umol/mmol creatinineAdult (>18 years old)BothHyperlysinuria details
    UrineDetected and Quantified16.110-51 umol/mmol creatinineChildren (1 - 13 years old)BothLysinuric protein intolerance details
    UrineDetected and Quantified332.7 umol/mmol creatinineAdult (>18 years old)Both
    Cystinuria
    details
    UrineDetected and Quantified4.106-13.859 umol/mmol creatinineAdult (>18 years old)BothHyperdibasic aminoaciduria I details
    UrineDetected and Quantified8.897-14.0297 umol/mmol creatinineChildren (1 - 13 years old)FemaleHyperdibasic aminoaciduria I details
    UrineDetected and Quantified92.570-123.800 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedFumaric aciduria details
    UrineDetected and Quantified3.371 +/- 6.3791 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified2.8009 +/- 3.4795 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Gastroesophageal reflux disease
      • Mordechai, Hien, ...
    details
    Associated Disorders and Diseases
    Disease References
    Alzheimer's disease
    1. Fonteh AN, Harrington RJ, Tsai A, Liao P, Harrington MG: Free amino acid and dipeptide changes in the body fluids from Alzheimer's disease subjects. Amino Acids. 2007 Feb;32(2):213-24. Epub 2006 Oct 10. [PubMed:17031479 ]
    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 ]
    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 ]
    Perillyl alcohol administration for cancer treatment
    1. Sugimoto M, Wong DT, Hirayama A, Soga T, Tomita M: Capillary electrophoresis mass spectrometry-based saliva metabolomics identified oral, breast and pancreatic cancer-specific profiles. Metabolomics. 2010 Mar;6(1):78-95. Epub 2009 Sep 10. [PubMed:20300169 ]
    Colorectal cancer
    1. 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. 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 ]
    3. 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. 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 ]
    Fumarase deficiency
    1. Allegri G, Fernandes MJ, Scalco FB, Correia P, Simoni RE, Llerena JC Jr, de Oliveira ML: Fumaric aciduria: an overview and the first Brazilian case report. J Inherit Metab Dis. 2010 Aug;33(4):411-9. doi: 10.1007/s10545-010-9134-2. Epub 2010 Jun 15. [PubMed:20549362 ]
    Hyperornithinemia with Gyrate Atrophy
    1. Simell O, Takki K: Raised plasma-ornithine and gyrate atrophy of the choroid and retina. Lancet. 1973 May 12;1(7811):1031-3. [PubMed:4122112 ]
    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. 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 ]
    2. 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 ]
    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..
    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.
    Ornithine transcarbamylase deficiency
    1. Harada E, Nishiyori A, Tokunaga Y, Watanabe Y, Kuriya N, Kumashiro R, Kuno T, Kuromaru R, Hirose S, Ichikawa K, Yoshino M: Late-onset ornithine transcarbamylase deficiency in male patients: prognostic factors and characteristics of plasma amino acid profile. Pediatr Int. 2006 Apr;48(2):105-11. [PubMed:16635166 ]
    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 ]
    Uremia
    1. Canepa A, Filho JC, Gutierrez A, Carrea A, Forsberg AM, Nilsson E, Verrina E, Perfumo F, Bergstrom J: Free amino acids in plasma, red blood cells, polymorphonuclear leukocytes, and muscle in normal and uraemic children. Nephrol Dial Transplant. 2002 Mar;17(3):413-21. [PubMed:11865086 ]
    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. 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. .
    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 ]
    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 ]
    Ulcerative colitis
    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 ]
    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 IDDB00129
    Phenol Explorer Compound IDNot Available
    FoodDB IDFDB003654
    KNApSAcK IDC00001384
    Chemspider ID6026
    KEGG Compound IDC00077
    BioCyc IDORNITHINE
    BiGG ID37976
    Wikipedia LinkOrnithine
    METLIN ID27
    PubChem Compound6262
    PDB IDORN
    ChEBI ID15729
    References
    Synthesis ReferenceZhang, Peng; Zhang, Shurong; Liu, Chunqiao; Yang, Yuhong. Method for preparing L-ornithine by enzymatic conversion. Faming Zhuanli Shenqing Gongkai Shuomingshu (2007), 8pp.
    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. 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 ]
    3. 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 ]
    4. 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 ]
    5. 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 ]
    6. Peng CT, Wu KH, Lan SJ, Tsai JJ, Tsai FJ, Tsai CH: Amino acid concentrations in cerebrospinal fluid in children with acute lymphoblastic leukemia undergoing chemotherapy. Eur J Cancer. 2005 May;41(8):1158-63. Epub 2005 Apr 14. [PubMed:15911239 ]
    7. Cynober LA: Plasma amino acid levels with a note on membrane transport: characteristics, regulation, and metabolic significance. Nutrition. 2002 Sep;18(9):761-6. [PubMed:12297216 ]
    8. Peters T, Thaete C, Wolf S, Popp A, Sedlmeier R, Grosse J, Nehls MC, Russ A, Schlueter V: A mouse model for cystinuria type I. Hum Mol Genet. 2003 Sep 1;12(17):2109-20. [PubMed:12923163 ]
    9. Mayer UM: [Hyperornithinaemia in patients with retinal dystrophy]. Ophthalmologe. 2003 Jan;100(1):55-61. [PubMed:12557027 ]
    10. Gray RG, Green A, Hall S, McKeown C: Prenatal exclusion of the HHH syndrome. Prenat Diagn. 1995 May;15(5):474-6. [PubMed:7644438 ]
    11. Jensen TG, Sullivan DM, Morgan RA, Taichman LB, Nussenblatt RB, Blaese RM, Csaky KG: Retrovirus-mediated gene transfer of ornithine-delta-aminotransferase into keratinocytes from gyrate atrophy patients. Hum Gene Ther. 1997 Nov 20;8(17):2125-32. [PubMed:9414260 ]
    12. Sell DR, Monnier VM: Ornithine is a novel amino acid and a marker of arginine damage by oxoaldehydes in senescent proteins. Ann N Y Acad Sci. 2005 Jun;1043:118-28. [PubMed:16037230 ]
    13. Stadler S, Gempel K, Bieger I, Pontz BF, Gerbitz KD, Bauer MF, Hofmann S: Detection of neonatal argininosuccinate lyase deficiency by serum tandem mass spectrometry. J Inherit Metab Dis. 2001 Jun;24(3):370-8. [PubMed:11486903 ]
    14. Gokmen SS, Aygit AC, Ayhan MS, Yorulmaz F, Gulen S: Significance of arginase and ornithine in malignant tumors of the human skin. J Lab Clin Med. 2001 May;137(5):340-4. [PubMed:11329531 ]
    15. Morizono H, Woolston JE, Colombini M, Tuchman M: The use of yeast mitochondria to study the properties of wild-type and mutant human mitochondrial ornithine transporter. Mol Genet Metab. 2005 Dec;86(4):431-40. Epub 2005 Oct 26. [PubMed:16256388 ]

    Only showing the first 10 proteins. There are 19 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
    References
    1. Gobert AP, Cheng Y, Wang JY, Boucher JL, Iyer RK, Cederbaum SD, Casero RA Jr, Newton JC, Wilson KT: Helicobacter pylori induces macrophage apoptosis by activation of arginase II. J Immunol. 2002 May 1;168(9):4692-700. [PubMed:11971019 ]
    2. Iwata S, Tsujino T, Ikeda Y, Ishida T, Ueyama T, Gotoh T, Mori M, Yokoyama M: Decreased expression of arginase II in the kidneys of Dahl salt-sensitive rats. Hypertens Res. 2002 May;25(3):411-8. [PubMed:12135320 ]
    3. Stickings P, Mistry SK, Boucher JL, Morris SM, Cunningham JM: Arginase expression and modulation of IL-1beta-induced nitric oxide generation in rat and human islets of Langerhans. Nitric Oxide. 2002 Dec;7(4):289-96. [PubMed:12446178 ]
    4. Bansal V, Ochoa JB: Arginine availability, arginase, and the immune response. Curr Opin Clin Nutr Metab Care. 2003 Mar;6(2):223-8. [PubMed:12589193 ]
    5. El Alami M, Dubois E, Oudjama Y, Tricot C, Wouters J, Stalon V, Messenguy F: Yeast epiarginase regulation, an enzyme-enzyme activity control: identification of residues of ornithine carbamoyltransferase and arginase responsible for enzyme catalytic and regulatory activities. J Biol Chem. 2003 Jun 13;278(24):21550-8. Epub 2003 Apr 4. [PubMed:12679340 ]
    General function:
    Involved in transaminase activity
    Specific function:
    Not Available
    Gene Name:
    OAT
    Uniprot ID:
    P04181
    Molecular weight:
    48534.39
    Reactions
    Ornithine + a 2-oxo acid → L-Glutamic gamma-semialdehyde + an L-amino aciddetails
    Ornithine + Oxoglutaric acid → L-Glutamic gamma-semialdehyde + L-Glutamic aciddetails
    References
    1. Levillain O, Balvay S, Peyrol S: Localization and differential expression of arginase II in the kidney of male and female mice. Pflugers Arch. 2005 Feb;449(5):491-503. Epub 2004 Dec 23. [PubMed:15616821 ]
    2. Inubushi T, Takasawa T, Tuboi Y, Watanabe N, Aki K, Katunuma N: Changes of glucose metabolism and skin-collagen neogenesis in vitamin B6 deficiency. Biofactors. 2005;23(2):59-67. [PubMed:16179747 ]
    3. Kaneko S, Ueda-Yamada M, Ando A, Matsumura S, Okuda-Ashitaka E, Matsumura M, Uyama M, Ito S: Cytotoxic effect of spermine on retinal pigment epithelial cells. Invest Ophthalmol Vis Sci. 2007 Jan;48(1):455-63. [PubMed:17197567 ]
    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
    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. Thomasset N, Quash GA, Dore JF: The differential contribution of arginase and transamidinase to ornithine biosynthesis in two achromic human melanoma cell lines. FEBS Lett. 1982 Nov 1;148(1):63-6. [PubMed:7173402 ]
    4. Natesan S, Reddy SR: Compensatory changes in enzymes of arginine metabolism during renal hypertrophy in mice. Comp Biochem Physiol B Biochem Mol Biol. 2001 Dec;130(4):585-95. [PubMed:11691635 ]
    5. Mitruka BM, Costilow RN: Arginine and ornithine catabolism by Clostridium botulinum. J Bacteriol. 1967 Jan;93(1):295-301. [PubMed:5335895 ]
    General function:
    Involved in metallopeptidase activity
    Specific function:
    Involved in the hydrolysis of N-acylated or N-acetylated amino acids (except L-aspartate).
    Gene Name:
    ACY1
    Uniprot ID:
    Q03154
    Molecular weight:
    45884.705
    Reactions
    N-Acetylornithine + Water → Acetic acid + Ornithinedetails
    General function:
    Involved in oxidoreductase activity
    Specific function:
    Bifunctional enzyme that converts glutamate to glutamate 5-semialdehyde, an intermediate in the biosynthesis of proline, ornithine and arginine.
    Gene Name:
    ALDH18A1
    Uniprot ID:
    P54886
    Molecular weight:
    87088.29
    General function:
    Involved in carboxyl- or carbamoyltransferase activity
    Specific function:
    Not Available
    Gene Name:
    OTC
    Uniprot ID:
    P00480
    Molecular weight:
    39934.775
    Reactions
    Carbamoyl phosphate + Ornithine → Phosphoric acid + Citrullinedetails
    References
    1. Barcelona-Andres B, Marina A, Rubio V: Gene structure, organization, expression, and potential regulatory mechanisms of arginine catabolism in Enterococcus faecalis. J Bacteriol. 2002 Nov;184(22):6289-300. [PubMed:12399499 ]
    2. El Alami M, Dubois E, Oudjama Y, Tricot C, Wouters J, Stalon V, Messenguy F: Yeast epiarginase regulation, an enzyme-enzyme activity control: identification of residues of ornithine carbamoyltransferase and arginase responsible for enzyme catalytic and regulatory activities. J Biol Chem. 2003 Jun 13;278(24):21550-8. Epub 2003 Apr 4. [PubMed:12679340 ]
    3. Templeton MD, Reinhardt LA, Collyer CA, Mitchell RE, Cleland WW: Kinetic analysis of the L-ornithine transcarbamoylase from Pseudomonas savastanoi pv. phaseolicola that is resistant to the transition state analogue (R)-N delta-(N'-sulfodiaminophosphinyl)-L-ornithine. Biochemistry. 2005 Mar 22;44(11):4408-15. [PubMed:15766270 ]
    4. Morizono H, Cabrera-Luque J, Shi D, Gallegos R, Yamaguchi S, Yu X, Allewell NM, Malamy MH, Tuchman M: Acetylornithine transcarbamylase: a novel enzyme in arginine biosynthesis. J Bacteriol. 2006 Apr;188(8):2974-82. [PubMed:16585758 ]
    General function:
    Involved in catalytic activity
    Specific function:
    Not Available
    Gene Name:
    ODC1
    Uniprot ID:
    P11926
    Molecular weight:
    51147.73
    Reactions
    Ornithine → Putrescine + CO(2)details
    Ornithine → Putrescine + Carbon dioxidedetails
    General function:
    Involved in arginase activity
    Specific function:
    May play a role in the regulation of extra-urea cycle arginine metabolism and also in down-regulation of nitric oxide synthesis. Extrahepatic arginase functions to regulate L-arginine bioavailability to NO synthase. Since NO synthase is found in the penile corpus cavernosum smooth muscle, the clitoral corpus cavernosum and the vagina, arginase II plays a role in both male and female sexual arousal. It is therefore a potential target for the treatment of male and female sexual arousal disorders.
    Gene Name:
    ARG2
    Uniprot ID:
    P78540
    Molecular weight:
    38577.515
    Reactions
    L-Arginine + Water → Ornithine + Ureadetails
    References
    1. Rodriguez PC, Zea AH, DeSalvo J, Culotta KS, Zabaleta J, Quiceno DG, Ochoa JB, Ochoa AC: L-arginine consumption by macrophages modulates the expression of CD3 zeta chain in T lymphocytes. J Immunol. 2003 Aug 1;171(3):1232-9. [PubMed:12874210 ]
    2. Levillain O, Hus-Citharel A, Garvi S, Peyrol S, Reymond I, Mutin M, Morel F: Ornithine metabolism in male and female rat kidney: mitochondrial expression of ornithine aminotransferase and arginase II. Am J Physiol Renal Physiol. 2004 Apr;286(4):F727-38. Epub 2004 Feb 10. [PubMed:14871882 ]
    3. Levillain O, Diaz JJ, Blanchard O, Dechaud H: Testosterone down-regulates ornithine aminotransferase gene and up-regulates arginase II and ornithine decarboxylase genes for polyamines synthesis in the murine kidney. Endocrinology. 2005 Feb;146(2):950-9. Epub 2004 Nov 11. [PubMed:15539552 ]
    4. Bussiere FI, Chaturvedi R, Cheng Y, Gobert AP, Asim M, Blumberg DR, Xu H, Kim PY, Hacker A, Casero RA Jr, Wilson KT: Spermine causes loss of innate immune response to Helicobacter pylori by inhibition of inducible nitric-oxide synthase translation. J Biol Chem. 2005 Jan 28;280(4):2409-12. Epub 2004 Nov 17. [PubMed:15548540 ]
    5. Nissim I, Luhovyy B, Horyn O, Daikhin Y, Nissim I, Yudkoff M: The role of mitochondrially bound arginase in the regulation of urea synthesis: studies with [U-15N4]arginine, isolated mitochondria, and perfused rat liver. J Biol Chem. 2005 May 6;280(18):17715-24. Epub 2005 Mar 7. [PubMed:15753084 ]
    General function:
    Involved in transport
    Specific function:
    Involved in the transport of the cationic amino acids (arginine, lysine and ornithine)
    Gene Name:
    SLC7A4
    Uniprot ID:
    O43246
    Molecular weight:
    68267.2
    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 transporter activity
    Specific function:
    Ornithine transport across inner mitochondrial membrane, from the cytoplasm to the matrix.
    Gene Name:
    SLC25A15
    Uniprot ID:
    Q9Y619
    Molecular weight:
    32735.96
    References
    1. Korman SH, Kanazawa N, Abu-Libdeh B, Gutman A, Tsujino S: Hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome with evidence of mitochondrial dysfunction due to a novel SLC25A15 (ORNT1) gene mutation in a Palestinian family. J Neurol Sci. 2004 Mar 15;218(1-2):53-8. [PubMed:14759633 ]
    2. Camacho JA, Mardach R, Rioseco-Camacho N, Ruiz-Pesini E, Derbeneva O, Andrade D, Zaldivar F, Qu Y, Cederbaum SD: Clinical and functional characterization of a human ORNT1 mutation (T32R) in the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome. Pediatr Res. 2006 Oct;60(4):423-9. Epub 2006 Aug 28. [PubMed:16940241 ]

    Only showing the first 10 proteins. There are 19 proteins in total.