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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-05-20 20:40:42 UTC
HMDB IDHMDB0000904
Secondary Accession Numbers
  • HMDB00904
Metabolite Identification
Common NameCitrulline
DescriptionCitrulline is an amino acid made from ornithine and carbamoyl phosphate in one of the central reactions in the urea cycle. It is also produced from arginine as a by-product of the reaction catalyzed by NOS family (NOS). In this reaction, arginine is first oxidized into N(omega)-hydroxyarginine, which is then further oxidized to citrulline concomitant with the release of nitric oxide (EC 1.14.13.39). Citrulline's name is derived from citrullus, the Latin word for watermelon, from which it was first isolated.
Structure
Thumb
Synonyms
ValueSource
(S)-2-amino-5-Ureidopentanoic acidChEBI
2-amino-5-Ureidovaleric acidChEBI
alpha-amino-delta-Ureidovaleric acidChEBI
CitChEBI
delta-UreidonorvalineChEBI
L-2-amino-5-Ureidovaleric acidChEBI
N(5)-(Aminocarbonyl)-L-ornithineChEBI
N5-(Aminocarbonyl)ornithineChEBI
N5-CarbamoylornithineChEBI
N(delta)-CarbamylornithineChEBI
(S)-2-amino-5-UreidopentanoateGenerator
2-amino-5-UreidovalerateGenerator
a-amino-delta-UreidovalerateGenerator
a-amino-delta-Ureidovaleric acidGenerator
alpha-amino-delta-UreidovalerateGenerator
α-amino-δ-ureidovalerateGenerator
α-amino-δ-ureidovaleric acidGenerator
δ-ureidonorvalineGenerator
L-2-amino-5-UreidovalerateGenerator
N(δ)-carbamylornithineGenerator
a-amino-δ-ureidovalerateGenerator
a-amino-δ-ureidovaleric acidGenerator
(2S)-2-amino-5-(carbamoylamino)PentanoateHMDB
(2S)-2-amino-5-(carbamoylamino)Pentanoic acidHMDB
(S)-2-amino-5-(Aminocarbonyl)aminopentanoateHMDB
(S)-2-amino-5-(Aminocarbonyl)aminopentanoic acidHMDB
2-amino-5-UredovalerateHMDB
2-amino-5-Uredovaleric acidHMDB
a-amino-D-UreidovalerateHMDB
a-amino-D-Ureidovaleric acidHMDB
alpha-amino-gamma-UreidovalerateHMDB
alpha-amino-gamma-Ureidovaleric acidHMDB
amino-UreidovalerateHMDB
amino-Ureidovaleric acidHMDB
CIRHMDB
CytrullineHMDB
D-UreidonorvalineHMDB
DL-CitrullineHMDB
GammaureidonorvalineHMDB
H-Cit-OHHMDB
L(+)-2-amino-5-UreidovalerateHMDB
L(+)-2-amino-5-Ureidovaleric acidHMDB
L(+)-CitrullineHMDB
L-2-amino-5-ureido-ValerateHMDB
L-2-amino-5-ureido-Valeric acidHMDB
L-CitrullineHMDB
L-CytrullineHMDB
L-N5-Carbamoyl-ornithineHMDB
N()-CarbamylornithineHMDB
N(5)-(Aminocarbonyl)-DL-ornithineHMDB
N-CarbamylornithineHMDB
N5-(Aminocarbonyl)-L-ornithineHMDB
N5-(Aminocarbonyl)-ornithineHMDB
N5-Carbamoyl-L-ornithineHMDB
N5-CarbamylornithineHMDB
ND-CarbamylornithineHMDB
Ndelta-carbamy-ornithineHMDB
Ndelta-carbamylornithineHMDB
Ngamma-carbamylornithineHMDB
SitrullineHMDB
UreidonorvalineHMDB
UreidovalerateHMDB
Ureidovaleric acidHMDB
Chemical FormulaC6H13N3O3
Average Molecular Weight175.1857
Monoisotopic Molecular Weight175.095691297
IUPAC Name(2S)-2-amino-5-(carbamoylamino)pentanoic acid
Traditional NameL-citrulline
CAS Registry Number372-75-8
SMILES
N[C@@H](CCCNC(N)=O)C(O)=O
InChI Identifier
InChI=1S/C6H13N3O3/c7-4(5(10)11)2-1-3-9-6(8)12/h4H,1-3,7H2,(H,10,11)(H3,8,9,12)/t4-/m0/s1
InChI KeyRHGKLRLOHDJJDR-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
  • Isourea
  • Amino acid
  • Carboximidic acid derivative
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Carboximidamide
  • Amine
  • Hydrocarbon derivative
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Organic oxide
  • Primary aliphatic amine
  • Organopnictogen compound
  • Imine
  • Organic oxygen compound
  • Organic nitrogen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Biological role:

Industrial application:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point235.5 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility212 mg/mLNot Available
LogP-3.19SANGSTER (1994)
Predicted Properties
PropertyValueSource
Water Solubility21.8 g/LALOGPS
logP-3.3ALOGPS
logP-3.9ChemAxon
logS-0.9ALOGPS
pKa (Strongest Acidic)2.27ChemAxon
pKa (Strongest Basic)9.23ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count4ChemAxon
Polar Surface Area118.44 ŲChemAxon
Rotatable Bond Count5ChemAxon
Refractivity41.33 m³·mol⁻¹ChemAxon
Polarizability17.35 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) (Non-derivatized)splash10-0a4i-0920000000-2d92b63cd5d9648023b8View in MoNA
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-00di-9610000000-2e7cd23afc2adcef35a3View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0a4i-0920000000-2d92b63cd5d9648023b8View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00di-9610000000-2e7cd23afc2adcef35a3View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-007o-9100000000-1f8dd2c6648b104639c7View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00dl-9410000000-37909012a777213f8566View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-008a-0904000000-23fbe48f82e515087d68View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-03di-5900000000-78afcbaf8b8b3eabf174View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-001i-0900000000-8fb191d4c20fd54b9282View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negativesplash10-00di-0900000000-da484f0362a8dca5127eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-00e9-0900000000-46229b4f77feabb3f857View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-001i-0900000000-4aca1022c393602a297dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-001i-0900000000-3bc2eff2e907b7734cc8View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-001i-3900000000-2613bf40e3be814da86fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0006-9300000000-e83287bbc060eb9cf6f3View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-001i-0900000000-daf5b8d935c6f60c6df7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00e9-0900000000-46229b4f77feabb3f857View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-001i-0900000000-4aca1022c393602a297dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-001i-0900000000-322d7f0082e7d5c6ebeeView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-001i-3900000000-2613bf40e3be814da86fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0006-9300000000-e83287bbc060eb9cf6f3View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT , negativesplash10-03di-5900000000-78afcbaf8b8b3eabf174View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT , negativesplash10-001i-0900000000-8fb191d4c20fd54b9282View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT , negativesplash10-00di-0900000000-da484f0362a8dca5127eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , negativesplash10-001i-0900000000-daf5b8d935c6f60c6df7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Linear Ion Trap , negativesplash10-001i-0900000000-20fe8593ca8d8303d73aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Linear Ion Trap , negativesplash10-001i-0900000000-a2851fcef80bb0d9b984View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0a4i-0900000000-4c1d7af748a47e489949View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00di-9000000000-988fced362fc0da157c9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-00di-9000000000-0818e0e8bcee12692498View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positivesplash10-004j-0900000000-5fa8a338dcd2f2a6bdd2View in MoNA
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,1H] 2D NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Sweat
  • Urine
Tissue Locations
  • Bladder
  • Epidermis
  • Fibroblasts
  • Intestine
  • Kidney
  • Liver
  • Myelin
  • Nerve Cells
  • Neuron
  • Placenta
  • Platelet
  • Prostate
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified10-60 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified6-40 uMNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified6-60 uMInfant (1 - 3 months old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified8-70 uMChildren (3 months - 6 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified10-70 uMChildren (6 - 18 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified19-51 uMInfant (0-1 year old)MaleNormal details
BloodDetected and Quantified38.0 (30.0-46.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified19-33 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified<43 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified8-47 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified10-45 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified10-50 uMChildren (1 - 13 years old)BothNormal details
BloodDetected and Quantified16 +/- 5 uMInfant (0-1 year old)BothNormal details
BloodDetected and Quantified29.8 +/- 7.91 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified35 +/- 10 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified31 +/- 8 uMChildren (1 - 13 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified20.4(16.7-26.3) uMChildren (1-13 uears old)Both
Normal
details
BloodDetected and Quantified36.0 +/- 8.0 uMChildren (1-13 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified37.0 +/- 9.0 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified35.0 +/- 10.0 uMAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified37 +/- 9 uMAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified308 uMChildren (1 - 13 years old)MaleNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified38.0 (30.0-46.0) uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified15.0-45.0 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified26 uMChildren (1-13 years old)Not SpecifiedNormal details
BloodDetected and Quantified5-37 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified10-60 uMChildren (1-13 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified2.8 +/- 1.0 uMAdult (>18 years old)MaleNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified2.8 +/- 1.8 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified2.62 (1.35-3.89) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified5.81+/- 1.23 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0-4.4 uMChildren (0 - 10 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0-4.5 uMAdolescent (>11 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified1.5 +/- 0.5 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
FecesDetected but not Quantified Adult (>18 years old)MaleNormal details
FecesDetected but not Quantified Children (6 - 18 years old)Not SpecifiedNormal details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected and Quantified340 +/- 270 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified70 +/- 50 nmol/g wet fecesAdult (>18 years old)Both
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)BothNormal details
SalivaDetected and Quantified25.30 +/- 19.00 uMAdult (>18 years old)FemaleNormal details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected and Quantified8.1 +/- 0.94 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified8.1 +/- 1.4 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified26.2 +/- 33.1 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified9.2 +/- 2.1 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified51.82 +/- 64.25 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected and Quantified6.8 +/- 4.1 uMAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified4.61 +/- 2.82 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SweatDetected and Quantified< 10 uMAdult (60 years old)Male
Normal
details
SweatDetected and Quantified< 10 uMAdult (40 years old)Male
Normal
details
UrineDetected and Quantified1.08 (0.065-2.1) umol/mmol creatinineAdult (>18 years old)BothNormal
    details
    UrineDetected and Quantified0.19 umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected but not Quantified Adult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified0.00-74.25 umol/mmol creatinineAdult (>18 years old)BothNormal
      • David F. Putnam C...
    details
    UrineDetected and Quantified0-16.507 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedNormal details
    UrineDetected and Quantified0.50 +/- 0.54 umol/mmol creatinineChildren (1-13 years old)MaleNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified1.1 +/- 0.84 umol/mmol creatinineAdult (>18 years old)MaleNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified0.99 +/- 0.69 umol/mmol creatinineAdult (>18 years old)FemaleNormal
      • Geigy Scientific ...
      • West Cadwell, N.J...
      • Basel, Switzerlan...
    details
    UrineDetected and Quantified0.24 +/- 0.23 umol/mmol creatinineAdult (>18 years old)BothNormal details
    UrineDetected and Quantified0.8 (0.2-1.7) umol/mmol creatinineAdult (>18 years old)Both
    Normal
    details
    UrineDetected and Quantified28.753 +/- 22.885 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Normal
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified0 - 10.97 umol/mmol creatinineNewborn (0-30 days old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 9.95 umol/mmol creatinineInfant (1 - 6 months old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 8.03 umol/mmol creatinineInfant (6 months - <1 year old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 7.01 umol/mmol creatinineChildren (1 - 2 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 5.99 umol/mmol creatinineChildren (2 - 4 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 4.97 umol/mmol creatinineChildren (4 - 13 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 3.96 umol/mmol creatinineAdolescent (13 - 21 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified0 - 3.96 umol/mmol creatinineAdult (>21 years old)Both
    Normal
      • BC Children's Hos...
    details
    UrineDetected and Quantified1.4114 +/- 1.0599 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Normal
      • Mordechai, Hien, ...
    details
    Abnormal Concentrations
    BiospecimenStatusValueAgeSexConditionReferenceDetails
    BloodDetected and Quantified15 uMChildren (1-13 years old)MalePearson Syndrome details
    BloodDetected and Quantified2 uMInfant (0-1 year old)FemalePearson Syndrome details
    BloodDetected and Quantified284 uMInfant (0-1 year old)MaleArgininosuccinic aciduria (ASL) details
    BloodDetected and Quantified27.5 (10.0-45.0) uMAdult (>18 years old)BothArgininosuccinic aciduria
      • MetaGene: Metabol...
    details
    BloodDetected and Quantified200.0 (100.0-300.0) uMChildren (1-13 years old)BothArgininosuccinic aciduria (ASL)
      • MetaGene: Metabol...
    details
    BloodDetected and Quantified13.0 (6.0-20.0) uMAdult (>18 years old)BothPermanent intestinal failure details
    BloodDetected and Quantified25.4 (23.4-27.4) uMAdult (>18 years old)BothAcute seizures details
    BloodDetected and Quantified26.8 (25.4-28.2) uMAdult (>18 years old)Both
    Epilepsy
    details
    BloodDetected and Quantified26.9 (25.2-28.6) uMChildren (1-13 years old)Both
    Epilepsy
    details
    BloodDetected and Quantified13 uMNewborn (0-30 days old)Male
    Narp Syndrome
    details
    BloodDetected and Quantified3-5 uMChildren (1-13 years old)MaleNarp Syndrome details
    BloodDetected and Quantified0-19 uMInfant (0-1 year old)BothNarp Syndrome details
    BloodDetected and Quantified86-1542 uMAdult (>18 years old)BothCitrullinemia type II, adult-onset details
    BloodDetected and Quantified6-21 uMChildren (1-13 years old)BothPhosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic details
    BloodDetected and Quantified5 uMNewborn (0-30 days old)MalePhosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic details
    BloodDetected and Quantified20.3(16.1-26.6) uMChildren (1-13 uears old)Both
    Environmental enteric dysfunction
    details
    BloodDetected and Quantified181 uMInfant (0-1 year old)MaleFumaric aciduria details
    BloodDetected and Quantified0-7 uMNewborn (0-30 days old)MaleN-Acetylglutamate synthetase deficiency details
    BloodDetected and Quantified13-23 uMInfant (0-1 year old)Male
    N-Acetylglutamate synthetase deficiency
    details
    BloodDetected and Quantified152-2752 uMChildren (1 - 13 years old)BothCitrullinemia Type I details
    BloodDetected and Quantified94.97 +/- 11.18 uMElderly (>65 years old)BothAlzheimer's disease details
    BloodDetected and Quantified28(0-275) uMChildren (1-13 years old)Female
    Pearson Syndrome
    details
    BloodDetected and Quantified286-485 uMNewborn (0-30 days old)BothCitrullinemia type II, neonatal-onset details
    BloodDetected and Quantified6 +/- 5 uMChildren (1-13 years old)BothCutis laxa, autosomal recessive, type IIIA details
    Cerebrospinal Fluid (CSF)Detected and Quantified27.0 +/- 2.6 uMAdult (>18 years old)BothAlzheimer's disease details
    Cerebrospinal Fluid (CSF)Detected and Quantified5.38 +/- 0.94 uMAdult (>18 years old)BothSchizophrenia details
    FecesDetected but not Quantified Adult (>18 years old)MaleGout details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Colorectal cancer
    details
    FecesDetected but not Quantified Adult (>18 years old)Femaleankylosing spondylitis details
    FecesDetected but not Quantified Adult (>18 years old)Femalerheumatoid arthritis 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)BothColorectal Cancer details
    FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
    SalivaDetected but not Quantified Adult (>18 years old)BothOral cancer details
    SalivaDetected but not Quantified Adult (>18 years old)FemaleBreast cancer details
    SalivaDetected but not Quantified Adult (>18 years old)Not SpecifiedPancreatic cancer details
    SalivaDetected but not Quantified Adult (>18 years old)Not SpecifiedPeriodontal diseases details
    SalivaDetected and Quantified17.64 +/- 10.31 uMAdult (>18 years old)MaleAlzheimer's disease details
    SalivaDetected and Quantified17.48 +/- 14.00 uMAdult (>18 years old)MaleFrontotemporal lobe dementia details
    SalivaDetected and Quantified14.67 +/- 10.99 uMAdult (>18 years old)BothLewy body disease details
    UrineDetected and Quantified63.800-549.400 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedFumaric aciduria details
    UrineDetected and Quantified21.0 +/- 2.8 umol/mmol creatinineAdult (>18 years old)BothAlzheimer's disease details
    UrineDetected and Quantified11.851 +/- 17.07 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified1.2423 +/- 1.4785 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    Eosinophilic esophagitis
      • Mordechai, Hien, ...
    details
    UrineDetected and Quantified1.0274 +/- 0.8653 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 ]
    Argininosuccinic aciduria
    1. Kleijer WJ, Garritsen VH, Linnebank M, Mooyer P, Huijmans JG, Mustonen A, Simola KO, Arslan-Kirchner M, Battini R, Briones P, Cardo E, Mandel H, Tschiedel E, Wanders RJ, Koch HG: Clinical, enzymatic, and molecular genetic characterization of a biochemical variant type of argininosuccinic aciduria: prenatal and postnatal diagnosis in five unrelated families. J Inherit Metab Dis. 2002 Sep;25(5):399-410. [PubMed:12408190 ]
    2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
    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 ]
    Citrullinemia type I
    1. Kose E, Unal O, Bulbul S, Gunduz M, Haberle J, Arslan N: Identification of three novel mutations in fourteen patients with citrullinemia type 1. Clin Biochem. 2017 Aug;50(12):686-689. doi: 10.1016/j.clinbiochem.2017.01.011. Epub 2017 Jan 27. [PubMed:28132756 ]
    Colorectal cancer
    1. 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 ]
    2. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
    3. 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 ]
    Epilepsy
    1. Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
    Schizophrenia
    1. Do KQ, Lauer CJ, Schreiber W, Zollinger M, Gutteck-Amsler U, Cuenod M, Holsboer F: gamma-Glutamylglutamine and taurine concentrations are decreased in the cerebrospinal fluid of drug-naive patients with schizophrenic disorders. J Neurochem. 1995 Dec;65(6):2652-62. [PubMed:7595563 ]
    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 ]
    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 ]
    Intestinal failure
    1. Crenn P, Coudray-Lucas C, Thuillier F, Cynober L, Messing B: Postabsorptive plasma citrulline concentration is a marker of absorptive enterocyte mass and intestinal failure in humans. Gastroenterology. 2000 Dec;119(6):1496-505. [PubMed:11113071 ]
    Rheumatoid arthritis
    1. Tie-juan ShaoZhi-xing HeZhi-jun XieHai-chang LiMei-jiao WangCheng-ping Wen. Characterization of ankylosing spondylitis and rheumatoid arthritis using 1H NMR-based metabolomics of human fecal extracts. Metabolomics. April 2016, 12:70 [Link]
    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 ]
    Gout
    1. Shao T, Shao L, Li H, Xie Z, He Z, Wen C: Combined Signature of the Fecal Microbiome and Metabolome in Patients with Gout. Front Microbiol. 2017 Feb 21;8:268. doi: 10.3389/fmicb.2017.00268. eCollection 2017. [PubMed:28270806 ]
    Eosinophilic esophagitis
    1. (). Mordechai, Hien, and David S. Wishart. .
    Citrullinemia type II, adult-onset
    1. Komatsu M, Yazaki M, Tanaka N, Sano K, Hashimoto E, Takei Y, Song YZ, Tanaka E, Kiyosawa K, Saheki T, Aoyama T, Kobayashi K: Citrin deficiency as a cause of chronic liver disorder mimicking non-alcoholic fatty liver disease. J Hepatol. 2008 Nov;49(5):810-20. doi: 10.1016/j.jhep.2008.05.016. Epub 2008 Jun 10. [PubMed:18620775 ]
    Citrullinemia type II, neonatal-onset
    1. Ohura T, Kobayashi K, Tazawa Y, Nishi I, Abukawa D, Sakamoto O, Iinuma K, Saheki T: Neonatal presentation of adult-onset type II citrullinemia. Hum Genet. 2001 Feb;108(2):87-90. [PubMed:11281457 ]
    Cutis laxa, autosomal recessive, type IIIA
    1. Baumgartner MR, Hu CA, Almashanu S, Steel G, Obie C, Aral B, Rabier D, Kamoun P, Saudubray JM, Valle D: Hyperammonemia with reduced ornithine, citrulline, arginine and proline: a new inborn error caused by a mutation in the gene encoding delta(1)-pyrroline-5-carboxylate synthase. Hum Mol Genet. 2000 Nov 22;9(19):2853-8. [PubMed:11092761 ]
    Myopathy, lactic acidosis, and sideroblastic anemia 1
    1. Parfait B, de Lonlay P, von Kleist-Retzow JC, Cormier-Daire V, Chretien D, Rotig A, Rabier D, Saudubray JM, Rustin P, Munnich A: The neurogenic weakness, ataxia and retinitis pigmentosa (NARP) syndrome mtDNA mutation (T8993G) triggers muscle ATPase deficiency and hypocitrullinaemia. Eur J Pediatr. 1999 Jan;158(1):55-8. [PubMed:9950309 ]
    Pearson Syndrome
    1. Crippa BL, Leon E, Calhoun A, Lowichik A, Pasquali M, Longo N: Biochemical abnormalities in Pearson syndrome. Am J Med Genet A. 2015 Mar;167A(3):621-8. doi: 10.1002/ajmg.a.36939. [PubMed:25691415 ]
    Phosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic
    1. Vieira P, Cameron J, Rahikkala E, Keski-Filppula R, Zhang LH, Santra S, Matthews A, Myllynen P, Nuutinen M, Moilanen JS, Rodenburg RJ, Rolfs A, Uusimaa J, van Karnebeek CDM: Novel homozygous PCK1 mutation causing cytosolic phosphoenolpyruvate carboxykinase deficiency presenting as childhood hypoglycemia, an abnormal pattern of urine metabolites and liver dysfunction. Mol Genet Metab. 2017 Apr;120(4):337-341. doi: 10.1016/j.ymgme.2017.02.003. Epub 2017 Feb 6. [PubMed:28216384 ]
    2. Santra S, Cameron JM, Shyr C, Zhang L, Drogemoller B, Ross CJ, Wasserman WW, Wevers RA, Rodenburg RJ, Gupte G, Preece MA, van Karnebeek CD: Cytosolic phosphoenolpyruvate carboxykinase deficiency presenting with acute liver failure following gastroenteritis. Mol Genet Metab. 2016 May;118(1):21-7. doi: 10.1016/j.ymgme.2016.03.001. Epub 2016 Mar 4. [PubMed:26971250 ]
    Ulcerative colitis
    1. 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 ]
    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
    • 104300 (Alzheimer's disease)
    • 207900 (Argininosuccinic aciduria)
    • 606812 (Fumarase deficiency)
    • 608300 (N-acetylglutamate synthetase deficiency)
    • 215700 (Citrullinemia type I)
    • 603471 (Citrullinemia type II, adult-onset)
    • 557000 (Pearson Syndrome)
    • 605814 (Citrullinemia type II, neonatal-onset)
    • 219150 (Cutis laxa, autosomal recessive, type IIIA)
    • 600462 (Myopathy, lactic acidosis, and sideroblastic anemia 1)
    • 261680 (Phosphoenolpyruvate Carboxykinase Deficiency 1, Cytosolic)
    • 181500 (Schizophrenia)
    • 114500 (Colorectal cancer)
    • 266600 (Crohn's disease)
    • 138900 (Gout)
    • 180300 (Rheumatoid arthritis)
    • 260350 (Pancreatic cancer)
    • 170650 (Periodontal disease)
    • 600274 (Frontotemporal dementia)
    • 610247 (Eosinophilic esophagitis)
    DrugBank IDDB00155
    Phenol Explorer Compound IDNot Available
    FoodDB IDFDB011841
    KNApSAcK IDC00001348
    Chemspider ID9367
    KEGG Compound IDC00327
    BioCyc IDL-CITRULLINE
    BiGG ID34627
    Wikipedia LinkCitrulline
    METLIN ID16
    PubChem Compound9750
    PDB IDCIR
    ChEBI ID16349
    References
    Synthesis ReferenceKakimoto, Toshio; Shibatani, Takeji; Nishimura, Noriyuki; Chibata, Ichiro. Enzymic production of L-citrulline by Pseudomonas putida. Applied Microbiology (1971), 22(6), 992-9.
    Material Safety Data Sheet (MSDS)Download (PDF)
    General References
    1. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
    2. Engelborghs S, Marescau B, De Deyn PP: Amino acids and biogenic amines in cerebrospinal fluid of patients with Parkinson's disease. Neurochem Res. 2003 Aug;28(8):1145-50. [PubMed:12834252 ]
    3. Hagenfeldt L, Bjerkenstedt L, Edman G, Sedvall G, Wiesel FA: Amino acids in plasma and CSF and monoamine metabolites in CSF: interrelationship in healthy subjects. J Neurochem. 1984 Mar;42(3):833-7. [PubMed:6198473 ]
    4. 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 ]
    5. Rainesalo S, Keranen T, Palmio J, Peltola J, Oja SS, Saransaari P: Plasma and cerebrospinal fluid amino acids in epileptic patients. Neurochem Res. 2004 Jan;29(1):319-24. [PubMed:14992292 ]
    6. Le Boucher J, Charret C, Coudray-Lucas C, Giboudeau J, Cynober L: Amino acid determination in biological fluids by automated ion-exchange chromatography: performance of Hitachi L-8500A. Clin Chem. 1997 Aug;43(8 Pt 1):1421-8. [PubMed:9267323 ]
    7. Crenn P, Coudray-Lucas C, Thuillier F, Cynober L, Messing B: Postabsorptive plasma citrulline concentration is a marker of absorptive enterocyte mass and intestinal failure in humans. Gastroenterology. 2000 Dec;119(6):1496-505. [PubMed:11113071 ]
    8. Melis GC, Boelens PG, van der Sijp JR, Popovici T, De Bandt JP, Cynober L, van Leeuwen PA: The feeding route (enteral or parenteral) affects the plasma response of the dipetide Ala-Gln and the amino acids glutamine, citrulline and arginine, with the administration of Ala-Gln in preoperative patients. Br J Nutr. 2005 Jul;94(1):19-26. [PubMed:16115328 ]
    9. DeLong GR, Glick TH: Ammonia metabolism in Reye syndrome and the effect of citrulline. Ann Neurol. 1982 Jan;11(1):53-8. [PubMed:7059128 ]
    10. Jianfeng G, Weiming Z, Ning L, Fangnan L, Li T, Nan L, Jieshou L: Serum citrulline is a simple quantitative marker for small intestinal enterocytes mass and absorption function in short bowel patients. J Surg Res. 2005 Aug;127(2):177-82. [PubMed:15921697 ]
    11. Liappis N, Pohl B, Weber HP, el-Karkani H: [Free amino acids in the saliva of children with phenylketonuria]. Klin Padiatr. 1986 Jan-Feb;198(1):25-8. [PubMed:3959486 ]
    12. Fleisher LD, Harris CJ, Mitchell DA, Nadler HL: Citrullinemia: prenatal diagnosis of an affected fetus. Am J Hum Genet. 1983 Jan;35(1):85-90. [PubMed:6823975 ]
    13. Potter MA, Zeesman S, Brennan B, Kobayashi K, Gao HZ, Tabata A, Saheki T, Whelan DT: Pregnancy in a healthy woman with untreated citrullinemia. Am J Med Genet A. 2004 Aug 15;129A(1):77-82. [PubMed:15266621 ]
    14. Wheatley DN, Kilfeather R, Stitt A, Campbell E: Integrity and stability of the citrulline-arginine pathway in normal and tumour cell lines. Cancer Lett. 2005 Sep 28;227(2):141-52. [PubMed:16112417 ]
    15. Reparon-Schuijt CC, van Esch WJ, van Kooten C, Schellekens GA, de Jong BA, van Venrooij WJ, Breedveld FC, Verweij CL: Secretion of anti-citrulline-containing peptide antibody by B lymphocytes in rheumatoid arthritis. Arthritis Rheum. 2001 Jan;44(1):41-7. [PubMed:11212174 ]
    16. Maruyama H, Ogawa M, Nishio T, Kobayashi K, Saheki T, Sunohara N: Citrullinemia type II in a 64-year-old man with fluctuating serum citrulline levels. J Neurol Sci. 2001 Jan 1;182(2):167-70. [PubMed:11137523 ]
    17. McLaurin J, Moscarello MA: The preparation of antibodies reactive against citrulline-containing charge isomers of myelin basic protein but not against the arginine-containing charge isomers. Anal Biochem. 1990 Dec;191(2):272-7. [PubMed:1707596 ]
    18. Facchinetti F, Longo M, Piccinini F, Neri I, Volpe A: L-arginine infusion reduces blood pressure in preeclamptic women through nitric oxide release. J Soc Gynecol Investig. 1999 Jul-Aug;6(4):202-7. [PubMed:10486782 ]
    19. Origuchi Y, Ushijima T, Sakaguchi M, Akaboshi I, Matsuda I: Citrullinemia presenting as uncontrollable epilepsy. Brain Dev. 1984;6(3):328-31. [PubMed:6486381 ]
    20. Booth FA, Haworth JC, Dilling LA, Perry TL, Greenberg CR, Seargeant LE, Penn AM, Rhead WJ: Mitochondrial encephalomyopathy with associated aminoacidopathy in a male sibship. J Pediatr. 1989 Jul;115(1):81-8. [PubMed:2738799 ]

    Only showing the first 10 proteins. There are 14 proteins in total.

    Enzymes

    General function:
    Involved in argininosuccinate synthase activity
    Specific function:
    Not Available
    Gene Name:
    ASS1
    Uniprot ID:
    P00966
    Molecular weight:
    46530.055
    Reactions
    Adenosine triphosphate + Citrulline + L-Aspartic acid → Adenosine monophosphate + Pyrophosphate + Argininosuccinic aciddetails
    Adenosine triphosphate + Citrulline + L-Aspartic acid → Adenosine monophosphate + Pyrophosphate + Argininosuccinic aciddetails
    References
    1. Braissant O, Honegger P, Loup M, Iwase K, Takiguchi M, Bachmann C: Hyperammonemia: regulation of argininosuccinate synthetase and argininosuccinate lyase genes in aggregating cell cultures of fetal rat brain. Neurosci Lett. 1999 May 7;266(2):89-92. [PubMed:10353334 ]
    2. Braissant O, Gotoh T, Loup M, Mori M, Bachmann C: L-arginine uptake, the citrulline-NO cycle and arginase II in the rat brain: an in situ hybridization study. Brain Res Mol Brain Res. 1999 Jul 5;70(2):231-41. [PubMed:10407171 ]
    3. Keilhoff G, Reiser M, Stanarius A, Aoki E, Wolf G: Citrulline immunohistochemistry for demonstration of NOS activity in vivo and in vitro. Nitric Oxide. 2000 Aug;4(4):343-53. [PubMed:10944418 ]
    4. Zhang B, Cao GL, Domachowske J, Jackson MJ, Porasuphatana S, Rosen GM: Stable expression of varied levels of inducible nitric oxide synthase in primary cultures of endothelial cells. Anal Biochem. 2000 Nov 15;286(2):198-205. [PubMed:11067741 ]
    5. Zhang WY, Gotoh T, Oyadomari S, Mori M: Coinduction of inducible nitric oxide synthase and arginine recycling enzymes in cytokine-stimulated PC12 cells and high output production of nitric oxide. Brain Res Mol Brain Res. 2000 Nov 10;83(1-2):1-8. [PubMed:11072090 ]
    General function:
    Involved in protein-arginine deiminase activity
    Specific function:
    Catalyzes the deimination of arginine residues of proteins. May be involved in cytoskeletal reorganization in the egg and early embryo (By similarity).
    Gene Name:
    PADI6
    Uniprot ID:
    Q6TGC4
    Molecular weight:
    77726.735
    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 protein-arginine deiminase activity
    Specific function:
    Catalyzes the citrullination/deimination of arginine residues of proteins. Citrullinates histone H3 at 'Arg-8' and/or 'Arg-17' and histone H4 at 'Arg-3', which prevents their methylation by CARM1 and HRMT1L2/PRMT1 and represses transcription. Citrullinates EP300/P300 at 'Arg-2142', which favors its interaction with NCOA2/GRIP1.
    Gene Name:
    PADI4
    Uniprot ID:
    Q9UM07
    Molecular weight:
    74078.65
    References
    1. Wang Y, Wysocka J, Sayegh J, Lee YH, Perlin JR, Leonelli L, Sonbuchner LS, McDonald CH, Cook RG, Dou Y, Roeder RG, Clarke S, Stallcup MR, Allis CD, Coonrod SA: Human PAD4 regulates histone arginine methylation levels via demethylimination. Science. 2004 Oct 8;306(5694):279-83. Epub 2004 Sep 2. [PubMed:15345777 ]
    2. Wysocka J, Allis CD, Coonrod S: Histone arginine methylation and its dynamic regulation. Front Biosci. 2006 Jan 1;11:344-55. [PubMed:16146736 ]
    3. Yamamoto K, Yamada R: Genome-wide single nucleotide polymorphism analyses of rheumatoid arthritis. J Autoimmun. 2005;25 Suppl:12-5. Epub 2005 Nov 2. [PubMed:16271291 ]
    4. Chang X, Han J: Expression of peptidylarginine deiminase type 4 (PAD4) in various tumors. Mol Carcinog. 2006 Mar;45(3):183-96. [PubMed:16355400 ]
    5. Okazaki Y, Suzuki A, Sawada T, Ohtake-Yamanaka M, Inoue T, Hasebe T, Yamada R, Yamamoto K: Identification of citrullinated eukaryotic translation initiation factor 4G1 as novel autoantigen in rheumatoid arthritis. Biochem Biophys Res Commun. 2006 Mar 3;341(1):94-100. Epub 2006 Jan 6. [PubMed:16412378 ]
    General function:
    Involved in protein-arginine deiminase activity
    Specific function:
    Catalyzes the deimination of arginine residues of proteins.
    Gene Name:
    PADI3
    Uniprot ID:
    Q9ULW8
    Molecular weight:
    74742.705
    References
    1. Dong S, Kanno T, Yamaki A, Kojima T, Shiraiwa M, Kawada A, Mechin MC, Chavanas S, Serre G, Simon M, Takahara H: NF-Y and Sp1/Sp3 are involved in the transcriptional regulation of the peptidylarginine deiminase type III gene (PADI3) in human keratinocytes. Biochem J. 2006 Aug 1;397(3):449-59. [PubMed:16671893 ]
    2. Iida A, Nakamura Y: Identification of 45 novel SNPs in the 83-kb region containing peptidylarginine deiminase types 1 and 3 loci on chromosomal band 1p36.13. J Hum Genet. 2004;49(7):387-90. Epub 2004 May 19. [PubMed:15150696 ]
    3. Kanno T, Kawada A, Yamanouchi J, Yosida-Noro C, Yoshiki A, Shiraiwa M, Kusakabe M, Manabe M, Tezuka T, Takahara H: Human peptidylarginine deiminase type III: molecular cloning and nucleotide sequence of the cDNA, properties of the recombinant enzyme, and immunohistochemical localization in human skin. J Invest Dermatol. 2000 Nov;115(5):813-23. [PubMed:11069618 ]
    General function:
    Involved in protein-arginine deiminase activity
    Specific function:
    Catalyzes the deimination of arginine residues of proteins (By similarity).
    Gene Name:
    PADI2
    Uniprot ID:
    Q9Y2J8
    Molecular weight:
    75563.35
    References
    1. Dong S, Kojima T, Shiraiwa M, Mechin MC, Chavanas S, Serre G, Simon M, Kawada A, Takahara H: Regulation of the expression of peptidylarginine deiminase type II gene (PADI2) in human keratinocytes involves Sp1 and Sp3 transcription factors. J Invest Dermatol. 2005 May;124(5):1026-33. [PubMed:15854045 ]
    General function:
    Involved in protein-arginine deiminase activity
    Specific function:
    Catalyzes the deimination of arginine residues of proteins (By similarity).
    Gene Name:
    PADI1
    Uniprot ID:
    Q9ULC6
    Molecular weight:
    74664.97
    References
    1. Iida A, Nakamura Y: Identification of 45 novel SNPs in the 83-kb region containing peptidylarginine deiminase types 1 and 3 loci on chromosomal band 1p36.13. J Hum Genet. 2004;49(7):387-90. Epub 2004 May 19. [PubMed:15150696 ]
    General function:
    Involved in hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amidines
    Specific function:
    Hydrolyzes N(G),N(G)-dimethyl-L-arginine (ADMA) and N(G)-monomethyl-L-arginine (MMA) which act as inhibitors of NOS. Has therefore a role in the regulation of nitric oxide generation.
    Gene Name:
    DDAH1
    Uniprot ID:
    O94760
    Molecular weight:
    20189.135
    Reactions
    Asymmetric dimethylarginine + Water → Dimethylamine + Citrullinedetails
    References
    1. Mishima T, Hamada T, Ui-Tei K, Takahashi F, Miyata Y, Imaki J, Suzuki H, Yamashita K: Expression of DDAH1 in chick and rat embryos. Brain Res Dev Brain Res. 2004 Feb 20;148(2):223-32. [PubMed:14766200 ]
    2. Tran CT, Fox MF, Vallance P, Leiper JM: Chromosomal localization, gene structure, and expression pattern of DDAH1: comparison with DDAH2 and implications for evolutionary origins. Genomics. 2000 Aug 15;68(1):101-5. [PubMed:10950934 ]
    3. Arrigoni FI, Vallance P, Haworth SG, Leiper JM: Metabolism of asymmetric dimethylarginines is regulated in the lung developmentally and with pulmonary hypertension induced by hypobaric hypoxia. Circulation. 2003 Mar 4;107(8):1195-201. [PubMed:12615801 ]
    General function:
    Involved in hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amidines
    Specific function:
    Hydrolyzes N(G),N(G)-dimethyl-L-arginine (ADMA) and N(G)-monomethyl-L-arginine (MMA) which act as inhibitors of NOS. Has therefore a role in the regulation of nitric oxide generation.
    Gene Name:
    DDAH2
    Uniprot ID:
    O95865
    Molecular weight:
    29643.54
    Reactions
    Asymmetric dimethylarginine + Water → Dimethylamine + Citrullinedetails
    References
    1. Tran CT, Fox MF, Vallance P, Leiper JM: Chromosomal localization, gene structure, and expression pattern of DDAH1: comparison with DDAH2 and implications for evolutionary origins. Genomics. 2000 Aug 15;68(1):101-5. [PubMed:10950934 ]
    2. Tain YL, Baylis C: Determination of dimethylarginine dimethylaminohydrolase activity in the kidney. Kidney Int. 2007 Oct;72(7):886-9. Epub 2007 Jul 25. [PubMed:17653133 ]
    General function:
    Involved in oxidoreductase activity
    Specific function:
    Produces nitric oxide (NO) which is a messenger molecule with diverse functions throughout the body. In macrophages, NO mediates tumoricidal and bactericidal actions. Also has nitrosylase activity and mediates cysteine S-nitrosylation of cytoplasmic target proteins such COX2.
    Gene Name:
    NOS2
    Uniprot ID:
    P35228
    Molecular weight:
    131116.3
    Reactions
    L-Arginine + NADPH + Oxygen → Citrulline + Nitric oxide + NADP + Waterdetails
    NADPH + N(omega)-Hydroxyarginine + Oxygen + Hydrogen Ion → NADP + Nitric oxide + Citrulline + Waterdetails
    L-Arginine + Oxygen + NADPH + Hydrogen Ion → Nitric oxide + Citrulline + NADP + Waterdetails
    References
    1. Cunningham JM, Rayne RC: Radiochemical measurement of NOS activity by conversion of [14C]L-arginine to citrulline using HPLC separation. Methods Mol Biol. 1998;100:75-81. [PubMed:10906995 ]
    2. Keilhoff G, Reiser M, Stanarius A, Aoki E, Wolf G: Citrulline immunohistochemistry for demonstration of NOS activity in vivo and in vitro. Nitric Oxide. 2000 Aug;4(4):343-53. [PubMed:10944418 ]
    3. Conrad KP, Powers RW, Davis AK, Novak J: Citrulline is not the major product using the standard "NOS activity" assay on renal cortical homogenates. Am J Physiol Regul Integr Comp Physiol. 2002 Jan;282(1):R303-10. [PubMed:11742852 ]
    4. Knowles RG, Salter M: Measurement of NOS activity by conversion of radiolabeled arginine to citrulline using ion-exchange separation. Methods Mol Biol. 1998;100:67-73. [PubMed:10906994 ]
    5. Yi GB, McClendon D, Desaiah D, Goddard J, Lister A, Moffitt J, Meer RK, deShazo R, Lee KS, Rockhold RW: Fire ant venom alkaloid, isosolenopsin A, a potent and selective inhibitor of neuronal nitric oxide synthase. Int J Toxicol. 2003 Mar-Apr;22(2):81-6. [PubMed:12745988 ]
    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. Quintero MJ, Muro-Pastor AM, Herrero A, Flores E: Arginine catabolism in the cyanobacterium Synechocystis sp. Strain PCC 6803 involves the urea cycle and arginase pathway. J Bacteriol. 2000 Feb;182(4):1008-15. [PubMed:10648527 ]
    2. 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 ]

    Only showing the first 10 proteins. There are 14 proteins in total.