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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2021-09-14 15:46:13 UTC
HMDB IDHMDB0000214
Secondary Accession Numbers
  • HMDB0000818
  • HMDB0004984
  • HMDB00214
  • HMDB00818
  • HMDB04984
Metabolite Identification
Common NameOrnithine
DescriptionOrnithine or L-ornithine, also known as (S)-2,5-diaminopentanoic acid 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 basic compound. Ornithine is a non-proteinogenic amino acid that plays a role in the urea cycle. It is considered to be a non-essential amino acid. A non-essential amino acid is an amino acid that can be synthesized from central metabolic pathway intermediates in humans and is not required in the diet. L-Ornithine is one of the products of the action of the enzyme arginase on L-arginine, creating urea. Therefore, ornithine is a central part of the urea cycle, which allows for the disposal of excess nitrogen. Outside the human body, L-ornithine is abundant in a number of food items such as wild rice, 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. L-ornithine exists in all living species, from bacteria to plants to humans. L-Ornithine is also a precursor of citrulline and arginine. In order for ornithine that is 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 the enzyme known as 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 ). In humans, L-ornithine is involved in a number of other metabolic disorders, some of which include, ornithine transcarbamylase deficiency (OTC deficiency), argininemia, and guanidinoacetate methyltransferase deficiency (GAMT deficiency). Ornithine is abnormally accumulated in the body in ornithine transcarbamylase deficiency. Moreover, Ornithine is found to be associated with cystinuria, hyperdibasic aminoaciduria I, and lysinuric protein intolerance, which are inborn errors of metabolism. It has been claimed that ornithine improves athletic performance, has anabolic effects, has wound-healing effects, and is immuno-enhancing.
Structure
Data?1600189035
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-diaminovalerateHMDB
(S)-a,Δ-diaminovaleric acidHMDB
(+)-S-OrnithineHMDB
(S)-a,D-DiaminovalerateHMDB
(S)-a,D-Diaminovaleric acidHMDB
5-Amino-L-norvalineHMDB
L-(-)-OrnithineHMDB
L-OrnithineHMDB
2,5-Diaminopentanoic acidHMDB
Ornithine dihydrochloride, (L)-isomerHMDB
Ornithine hydrochloride, (D)-isomerHMDB
Ornithine, (D)-isomerHMDB
Ornithine, (L)-isomerHMDB
2,5 Diaminopentanoic acidHMDB
Ornithine monoacetate, (L)-isomerHMDB
Ornithine monohydrochloride, (D)-isomerHMDB
Ornithine monohydrochloride, (DL)-isomerHMDB
Ornithine phosphate (1:1), (L)-isomerHMDB
Ornithine sulfate (1:1), (L)-isomerHMDB
Ornithine hydrochloride, (DL)-isomerHMDB
Ornithine hydrochloride, (L)-isomerHMDB
Ornithine monohydrobromide, (L)-isomerHMDB
Ornithine, (DL)-isomerHMDB
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
Description 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 Molecular Properties
PropertyValueReference
Melting Point140 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility620 mg/mLNot Available
LogP-4.22SANGSTER (1994)
Predicted Molecular 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
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Spectral Properties

Collision Cross Sections

NameAdductTypeData SourceValueReference
DeepCCS[M-H]-ExperimentalAstarita_neg127.330932474
DeepCCS[M-H]-ExperimentalMetCCS_test_neg126.130932474
DeepCCS[M+H]+ExperimentalAstarita_pos121.330932474
AllCCS[M-H]-ExperimentalNot Available131.4http://allccs.zhulab.cn/database/detail?ID=AllCCS00000082
AllCCS[M+H]+ExperimentalNot Available128.1http://allccs.zhulab.cn/database/detail?ID=AllCCS00000082
DarkChem[M+H]+PredictedNot Available129.57131661259
DarkChem[M+H]+PredictedNot Available129.57131661259
DarkChem[M-H]-PredictedNot Available123.71131661259
DarkChem[M-H]-PredictedNot Available123.71131661259
AllCCS[M+H]+PredictedNot Available130.74532859911
AllCCS[M-H]-PredictedNot Available124.96632859911

Retention Indices

Underivatized

Not Available

Derivatized

DerivativeValueReference
Ornithine,1TMS,#11369.6937https://arxiv.org/abs/1905.12712
Ornithine,1TMS,#21525.6691https://arxiv.org/abs/1905.12712
Ornithine,1TMS,#31479.8158https://arxiv.org/abs/1905.12712
Ornithine,1TBDMS,#11600.4789https://arxiv.org/abs/1905.12712
Ornithine,1TBDMS,#21785.88https://arxiv.org/abs/1905.12712
Ornithine,1TBDMS,#31729.7065https://arxiv.org/abs/1905.12712
Spectra

GC-MS

Spectrum TypeDescriptionSplash KeyDeposition DateView
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-0006-1920000000-b0357b8c9888abda7d802014-06-16View Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0006-0910000000-8aaa0faabf1429b906112014-06-16View Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)splash10-00di-7910000000-5fa118444ceb45204aa32014-06-16View Spectrum
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-006x-3910000000-f6d0c6492f2cab33a3922014-06-16View Spectrum
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-00dr-2900000000-77222ff2d0118d3d3b572014-06-16View Spectrum
GC-MSGC-MS Spectrum - GC-MS (4 TMS)splash10-0006-1910000000-53c73c4fa3665c44d3b92014-06-16View Spectrum
GC-MSGC-MS Spectrum - GC-MS (5 TMS)splash10-03di-1691000000-dc14976cfee86cc828b92014-06-16View Spectrum
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0006-0920000000-f65f61e4a3739eabb1d12017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0006-1920000000-b0357b8c9888abda7d802017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0006-0910000000-8aaa0faabf1429b906112017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00di-7910000000-5fa118444ceb45204aa32017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-006x-3910000000-f6d0c6492f2cab33a3922017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00dr-2900000000-77222ff2d0118d3d3b572017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0006-1910000000-53c73c4fa3665c44d3b92017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-03di-1691000000-dc14976cfee86cc828b92017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0006-0900000000-f59681b9f46f1b7384872017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0006-0900000000-3048477975b51ae176af2017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00dr-2900000000-8d1a2cc0a88f7d9216e12017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0udi-1900000000-bc40b76e8b03ef7a08f02017-09-12View Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-001l-9000000000-50fe14c2cb68fd09e32f2016-09-22View Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-0089-9300000000-bbfdea03043e52ad8a152017-10-06View Spectrum
MSMass Spectrum (Electron Ionization)splash10-001u-9000000000-deb45bcb4280a1e67a8f2021-09-05View Spectrum
MSMass Spectrum (Electron Ionization)splash10-0a4i-8900000000-5b42adf83e422cffefb62021-09-18View Spectrum
MSMass Spectrum (Electron Ionization)splash10-0a4i-9800000000-24fa3ff739ff064312ab2021-09-18View Spectrum

LC-MS/MS

Spectrum TypeDescriptionSplash KeyDeposition DateView
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-01b9-6900000000-8fe592b77d2d8e4ae7042012-07-24View Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00di-9000000000-462b84db532c335275802012-07-24View Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-00di-9000000000-9c9c28d2e40d5eadacd22012-07-24View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-e44e06092c06bb4791af2012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-014i-9000000000-4d6ccc429f5155b246542012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-014i-0900000000-dcad699e8ed7e1b198602012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-7b130437d4e1ceea277b2012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-001i-0900000000-0fae230bc6b21623a4bf2012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-014i-9000000000-5a350a8e7c607a8b9a602012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-014i-0900000000-516054b6c2f3cd4a1d022012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-000i-0793321000-9fef988b5ce019f846422012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-03di-0900000000-a612df8df5f8ba1ff7f32012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-001i-0910000000-97fad20414656e2b7b932012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-01di-0490000000-1b137b937beb110d2e4b2012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-001i-0900000000-e3fc95eb909e45d8e6482012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-001i-1900000000-cdd9aac89a5a71814ff32012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-001r-9500000000-f1cc3eccf3f5ab7342222012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-0159-0900000000-93ce7b142d1f82f17e952012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-00di-9200000000-186f270471bdec40f8042012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-00di-9000000000-ebbdf0ab6d3180cba1a52012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-00di-9000000000-207bcc826fb2238c2cc52012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-00di-9000000000-a2f9a950fb23a6b188572012-08-31View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-01c9-9700000000-0364d35bb1a7c10a09c72016-09-12View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00dr-9100000000-9c4015733140885b49572016-09-12View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-00dl-9000000000-b32e7180e30a835008bb2016-09-12View Spectrum

NMR

Spectrum TypeDescriptionDeposition DateView
1D NMR1H NMR Spectrum (1D, 500 MHz, H2O, experimental)2012-12-04View Spectrum
1D NMR13C NMR Spectrum (1D, 400 MHz, H2O, experimental)2012-12-04View Spectrum
1D NMR1H NMR Spectrum (1D, D2O, experimental)2016-10-22View Spectrum
1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16View Spectrum
1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16View Spectrum
2D NMR[1H, 1H] 2D NMR Spectrum (predicted)2012-12-04View Spectrum
2D NMR[1H, 13C] NMR Spectrum (2D, 600 MHz, H2O, predicted)2012-12-05View Spectrum
Biological Properties
Cellular Locations
  • Extracellular
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Sweat
  • Urine
Tissue Locations
  • Epidermis
  • Intestine
  • Liver
  • Prostate
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 but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal 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 QuantifiedNot QuantifiedAdult (>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 QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedInfant (0-1 year old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedChildren (1-13 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedBoth
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedNormal details
FecesDetected and Quantified20 +/- 20 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedNot 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 QuantifiedNot QuantifiedAdult (>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 QuantifiedNot QuantifiedAdult 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 Quantified<1.92 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified1.44-24.36 umol/mmol creatinineNewborn (0-30 days old)BothNormal
    • López Hernández Y...
details
UrineDetected and Quantified4.72(2.03-10.45) umol/mmol creatinineNewborn (0-30 days old)FemaleNormal
    • López Hernández Y...
details
UrineDetected and Quantified5.79(1.28-25.46) umol/mmol creatinineNewborn (0-30 days old)MaleNormal
    • López Hernández Y...
details
UrineDetected and Quantified0.98 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified<2.036 umol/mmol creatinineAdolescent (13-18 years old)Not SpecifiedNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>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 Quantified<2.83 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal 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 and Quantified<29.97 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>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
      Not Available
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 Quantified3.34 (0-6.67) 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 Quantified<7.92 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal 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 Quantified0-17 umol/mmol creatinineInfant (0-1 year old)Both
Normal
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
UrineDetected and Quantified<4.98 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified31.66 +/- 29.40 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified54.27 +/- 47.49 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
UrineDetected and Quantified76.88 +/- 79.14 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
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 QuantifiedNot QuantifiedAdult (>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 QuantifiedNot QuantifiedAdult (>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 QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedChildren (1-13 years old)BothAutism details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)BothCrohns disease details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)BothUlcerative colitis details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedCrohns disease details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedUlcerative colitis details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedUnclassified IBD details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothOral cancer details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleBreast cancer details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not SpecifiedPancreatic cancer details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>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 but not QuantifiedNot QuantifiedAdult (>18 years old)BothBladder cancer 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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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..
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 ]
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 ]
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.
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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.
Eosinophilic esophagitis
  1. (). Mordechai, Hien, and David S. Wishart. .
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 ]
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 ]
Associated OMIM IDs
DrugBank IDDB00129
Phenol Explorer Compound IDNot Available
FooDB IDFDB030969
KNApSAcK IDC00001384
Chemspider ID6026
KEGG Compound IDC00077
BioCyc IDL-ORNITHINE
BiGG ID37976
Wikipedia LinkOrnithine
METLIN ID27
PubChem Compound6262
PDB IDNot Available
ChEBI ID15729
Food Biomarker OntologyNot Available
VMH IDORN
MarkerDB IDMDB00000102
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. Mayer UM: [Hyperornithinaemia in patients with retinal dystrophy]. Ophthalmologe. 2003 Jan;100(1):55-61. [PubMed:12557027 ]
  2. 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 ]
  3. 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 ]
  4. Gray RG, Green A, Hall S, McKeown C: Prenatal exclusion of the HHH syndrome. Prenat Diagn. 1995 May;15(5):474-6. [PubMed:7644438 ]
  5. 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 ]
  6. 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 ]
  7. 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 ]
  8. 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 ]
  9. 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 ]
  10. 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 ]
  11. 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 ]
  12. 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 ]
  13. 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 ]
  14. 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 ]
  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
N2-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 → Phosphate + 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.