You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.

Jump To Section:

Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (19) Show 19 proteins XML

Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2021-09-14 15:46:13 UTC

HMDB ID

HMDB0000214

Secondary Accession Numbers

HMDB0000818
HMDB0004984
HMDB00214
HMDB00818
HMDB04984

Metabolite Identification

Common Name

Ornithine

Description

Ornithine 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

MOL 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

COMPND    https://cactus.nci.nih.gov/chemical/structure/NCCC%5BC@H%5D(N)C(O)=O/file?for... 
AUTHOR    GENERATED BY OPEN BABEL 3.1.0
HETATM    1  N   UNL     1       4.135  -0.049  -0.078  1.00  0.00           N  
HETATM    2  C   UNL     1       2.853  -0.502   0.479  1.00  0.00           C  
HETATM    3  C   UNL     1       1.703   0.145  -0.294  1.00  0.00           C  
HETATM    4  C   UNL     1       0.369  -0.327   0.286  1.00  0.00           C  
HETATM    5  CA  UNL     1      -0.782   0.321  -0.488  1.00  0.00           C  
HETATM    6  HA  UNL     1      -0.670   0.108  -1.551  1.00  0.00           H  
HETATM    7  N   UNL     1      -0.758   1.774  -0.274  1.00  0.00           N  
HETATM    8  C   UNL     1      -2.093  -0.237   0.002  1.00  0.00           C  
HETATM    9  O   UNL     1      -2.518  -1.433  -0.435  1.00  0.00           O  
HETATM   10  OXT UNL     1      -2.762   0.392   0.787  1.00  0.00           O  
HETATM   11  H   UNL     1       4.911  -0.463   0.416  1.00  0.00           H  
HETATM   12  H   UNL     1       4.189  -0.245  -1.066  1.00  0.00           H  
HETATM   13  H   UNL     1       2.784  -1.587   0.393  1.00  0.00           H  
HETATM   14  H   UNL     1       2.792  -0.217   1.529  1.00  0.00           H  
HETATM   15  H   UNL     1       1.773   1.230  -0.208  1.00  0.00           H  
HETATM   16  H   UNL     1       1.765  -0.140  -1.344  1.00  0.00           H  
HETATM   17  H   UNL     1       0.299  -1.411   0.200  1.00  0.00           H  
HETATM   18  H   UNL     1       0.307  -0.041   1.336  1.00  0.00           H  
HETATM   19  H1  UNL     1      -1.468   2.232  -0.825  1.00  0.00           H  
HETATM   20  H2  UNL     1      -0.860   1.997   0.705  1.00  0.00           H  
HETATM   21  H   UNL     1      -3.365  -1.750  -0.093  1.00  0.00           H  
CONECT    1    2   11   12                                            
CONECT    2    1    3   13   14                                       
CONECT    3    2    4   15   16                                       
CONECT    4    3    5   17   18                                       
CONECT    5    4    6    7    8                                       
CONECT    6    5                                                      
CONECT    7    5   19   20                                            
CONECT    8    5    9   10   10                                       
CONECT    9    8   21                                                 
CONECT   10    8    8                                                 
CONECT   11    1                                                      
CONECT   12    1                                                      
CONECT   13    2                                                      
CONECT   14    2                                                      
CONECT   15    3                                                      
CONECT   16    3                                                      
CONECT   17    4                                                      
CONECT   18    4                                                      
CONECT   19    7                                                      
CONECT   20    7                                                      
CONECT   21    9                                                      
MASTER        0    0    0    0    0    0    0    0   21    0   21    0
END

View in JSmol View Stereo Labels

Synonyms

Value	Source
(S)-2,5-Diaminopentanoate	ChEBI
(S)-2,5-Diaminopentanoic acid	ChEBI
(S)-2,5-Diaminovaleric acid	ChEBI
(S)-alpha,delta-Diaminovaleric acid	ChEBI
(S)-Ornithine	ChEBI
(S)-2,5-Diaminovalerate	Generator
(S)-a,delta-Diaminovalerate	Generator
(S)-a,delta-Diaminovaleric acid	Generator
(S)-alpha,delta-Diaminovalerate	Generator
(S)-Α,δ-diaminovalerate	Generator
(S)-Α,δ-diaminovaleric acid	Generator
(S)-a,Δ-diaminovalerate	HMDB
(S)-a,Δ-diaminovaleric acid	HMDB
(+)-S-Ornithine	HMDB
(S)-a,D-Diaminovalerate	HMDB
(S)-a,D-Diaminovaleric acid	HMDB
5-Amino-L-norvaline	HMDB
L-(-)-Ornithine	HMDB
L-Ornithine	HMDB
2,5-Diaminopentanoic acid	HMDB
Ornithine dihydrochloride, (L)-isomer	HMDB
Ornithine hydrochloride, (D)-isomer	HMDB
Ornithine, (D)-isomer	HMDB
Ornithine, (L)-isomer	HMDB
2,5 Diaminopentanoic acid	HMDB
Ornithine monoacetate, (L)-isomer	HMDB
Ornithine monohydrochloride, (D)-isomer	HMDB
Ornithine monohydrochloride, (DL)-isomer	HMDB
Ornithine phosphate (1:1), (L)-isomer	HMDB
Ornithine sulfate (1:1), (L)-isomer	HMDB
Ornithine hydrochloride, (DL)-isomer	HMDB
Ornithine hydrochloride, (L)-isomer	HMDB
Ornithine monohydrobromide, (L)-isomer	HMDB
Ornithine, (DL)-isomer	HMDB

Chemical Formula

C₅H₁₂N₂O₂

Average Molecular Weight

132.161

Monoisotopic Molecular Weight

132.089877638

IUPAC Name

(2S)-2,5-diaminopentanoic acid

Traditional Name

ornithine

CAS Registry Number

3184-13-2

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 Key

AHLPHDHHMVZTML-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.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

L-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 Framework

Aliphatic acyclic compounds

External Descriptors

non-proteinogenic L-alpha-amino acid (CHEBI:15729 )
ornithine (CHEBI:15729 )
Other amino acids (C00077 )

Ontology

Leukemia

Disposition

Route of exposure:

Enteral:

Ingestion

Organ and components:

Biofluid and excreta:

Cell and elements:

Extracellular

Subcellular:

Process

Naturally occurring process:

Biological process:

Biochemical pathway:

Role

Industrial application:

Pharmaceutical industry:

Pharmaceutical

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	140 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	620 mg/mL	Not Available
LogP	-4.22	SANGSTER (1994)

Predicted Molecular Properties

Property	Value	Source
Water Solubility	172 g/L	ALOGPS
logP	-3.6	ALOGPS
logP	-3.7	ChemAxon
logS	0.11	ALOGPS
pKa (Strongest Acidic)	2.67	ChemAxon
pKa (Strongest Basic)	10.29	ChemAxon
Physiological Charge	1	ChemAxon
Hydrogen Acceptor Count	4	ChemAxon
Hydrogen Donor Count	3	ChemAxon
Polar Surface Area	89.34 Å²	ChemAxon
Rotatable Bond Count	4	ChemAxon
Refractivity	33.21 m³·mol⁻¹	ChemAxon
Polarizability	13.85 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Predicted Spectral Properties

Collision Cross Sections

Name	Adduct	Type	Data Source	Value	Reference
DeepCCS	[M-H]-	Experimental	Astarita_neg	127.3	30932474
DeepCCS	[M-H]-	Experimental	MetCCS_test_neg	126.1	30932474
DeepCCS	[M+H]+	Experimental	Astarita_pos	121.3	30932474
AllCCS	[M-H]-	Experimental	Not Available	131.4	http://allccs.zhulab.cn/database/detail?ID=AllCCS00000082
AllCCS	[M+H]+	Experimental	Not Available	128.1	http://allccs.zhulab.cn/database/detail?ID=AllCCS00000082
DarkChem	[M+H]+	Predicted	Not Available	129.571	31661259
DarkChem	[M+H]+	Predicted	Not Available	129.571	31661259
DarkChem	[M-H]-	Predicted	Not Available	123.711	31661259
DarkChem	[M-H]-	Predicted	Not Available	123.711	31661259
AllCCS	[M+H]+	Predicted	Not Available	130.745	32859911
AllCCS	[M-H]-	Predicted	Not Available	124.966	32859911

Retention Indices

Underivatized

Not Available

Derivatized

Derivative	Value	Reference
Ornithine,1TMS,#1	1369.6937	https://arxiv.org/abs/1905.12712
Ornithine,1TMS,#2	1525.6691	https://arxiv.org/abs/1905.12712
Ornithine,1TMS,#3	1479.8158	https://arxiv.org/abs/1905.12712
Ornithine,1TBDMS,#1	1600.4789	https://arxiv.org/abs/1905.12712
Ornithine,1TBDMS,#2	1785.88	https://arxiv.org/abs/1905.12712
Ornithine,1TBDMS,#3	1729.7065	https://arxiv.org/abs/1905.12712

Spectra

GC-MS

Spectrum Type	Description	Splash Key	Deposition Date	View
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-0006-1920000000-b0357b8c9888abda7d80	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)	splash10-0006-0910000000-8aaa0faabf1429b90611	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-00di-7910000000-5fa118444ceb45204aa3	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (3 TMS)	splash10-006x-3910000000-f6d0c6492f2cab33a392	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (3 TMS)	splash10-00dr-2900000000-77222ff2d0118d3d3b57	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (4 TMS)	splash10-0006-1910000000-53c73c4fa3665c44d3b9	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (5 TMS)	splash10-03di-1691000000-dc14976cfee86cc828b9	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - EI-B (Non-derivatized)	splash10-0006-0920000000-f65f61e4a3739eabb1d1	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0006-1920000000-b0357b8c9888abda7d80	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0006-0910000000-8aaa0faabf1429b90611	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-00di-7910000000-5fa118444ceb45204aa3	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-006x-3910000000-f6d0c6492f2cab33a392	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-00dr-2900000000-77222ff2d0118d3d3b57	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-0006-1910000000-53c73c4fa3665c44d3b9	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-03di-1691000000-dc14976cfee86cc828b9	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0006-0900000000-f59681b9f46f1b738487	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0006-0900000000-3048477975b51ae176af	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-00dr-2900000000-8d1a2cc0a88f7d9216e1	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0udi-1900000000-bc40b76e8b03ef7a08f0	2017-09-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-001l-9000000000-50fe14c2cb68fd09e32f	2016-09-22	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positive	splash10-0089-9300000000-bbfdea03043e52ad8a15	2017-10-06	View Spectrum
MS	Mass Spectrum (Electron Ionization)	splash10-001u-9000000000-deb45bcb4280a1e67a8f	2021-09-05	View Spectrum
MS	Mass Spectrum (Electron Ionization)	splash10-0a4i-8900000000-5b42adf83e422cffefb6	2021-09-18	View Spectrum
MS	Mass Spectrum (Electron Ionization)	splash10-0a4i-9800000000-24fa3ff739ff064312ab	2021-09-18	View Spectrum

LC-MS/MS

Spectrum Type	Description	Splash Key	Deposition Date	View
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)	splash10-01b9-6900000000-8fe592b77d2d8e4ae704	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)	splash10-00di-9000000000-462b84db532c33527580	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)	splash10-00di-9000000000-9c9c28d2e40d5eadacd2	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-001i-0900000000-e44e06092c06bb4791af	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-014i-9000000000-4d6ccc429f5155b24654	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-014i-0900000000-dcad699e8ed7e1b19860	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-001i-0900000000-7b130437d4e1ceea277b	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-001i-0900000000-0fae230bc6b21623a4bf	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-014i-9000000000-5a350a8e7c607a8b9a60	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive	splash10-014i-0900000000-516054b6c2f3cd4a1d02	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-000i-0793321000-9fef988b5ce019f84642	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-03di-0900000000-a612df8df5f8ba1ff7f3	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-001i-0910000000-97fad20414656e2b7b93	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-01di-0490000000-1b137b937beb110d2e4b	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative	splash10-001i-0900000000-e3fc95eb909e45d8e648	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative	splash10-001i-1900000000-cdd9aac89a5a71814ff3	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative	splash10-001r-9500000000-f1cc3eccf3f5ab734222	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positive	splash10-0159-0900000000-93ce7b142d1f82f17e95	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positive	splash10-00di-9200000000-186f270471bdec40f804	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positive	splash10-00di-9000000000-ebbdf0ab6d3180cba1a5	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positive	splash10-00di-9000000000-207bcc826fb2238c2cc5	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positive	splash10-00di-9000000000-a2f9a950fb23a6b18857	2012-08-31	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-01c9-9700000000-0364d35bb1a7c10a09c7	2016-09-12	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-00dr-9100000000-9c4015733140885b4957	2016-09-12	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-00dl-9000000000-b32e7180e30a835008bb	2016-09-12	View Spectrum

NMR

Spectrum Type	Description	Deposition Date	View
1D NMR	¹H NMR Spectrum (1D, 500 MHz, H₂O, experimental)	2012-12-04	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 400 MHz, H₂O, experimental)	2012-12-04	View Spectrum
1D NMR	¹H NMR Spectrum (1D, D₂O, experimental)	2016-10-22	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-16	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-16	View Spectrum
2D NMR	[¹H, ¹H] 2D NMR Spectrum (predicted)	2012-12-04	View Spectrum
2D NMR	[¹H, ¹³C] NMR Spectrum (2D, 600 MHz, H₂O, predicted)	2012-12-05	View Spectrum

Biological Properties

Cellular Locations

Extracellular
Mitochondria

Biospecimen Locations

Blood
Cerebrospinal Fluid (CSF)
Feces
Saliva
Sweat
Urine

Tissue Locations

Epidermis
Intestine
Liver
Prostate

Pathways

Name	SMPDB/Pathwhiz	KEGG
Arginine and Proline Metabolism
Urea Cycle
Spermidine and Spermine Biosynthesis		Not Available
Glycine and Serine Metabolism
Prolidase Deficiency (PD)		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	55.0 (39.0-71.0) uM	Adult (>18 years old)	Both	Normal	12297216	details
Blood	Detected and Quantified	93.8 +/- 41.3 uM	Adult (>18 years old)	Both	Normal	21359215	details
Blood	Detected and Quantified	27-86 uM	Adult (>18 years old)	Not Specified	Normal	Lysinuric Protein...	details
Blood	Detected and Quantified	90.0 +/- 20.0 uM	Newborn (0-30 days old)	Not Specified	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	47.0 +/- 14.0 uM	Children (1-13 years old)	Female	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	65.0 +/- 18.0 uM	Adult (>18 years old)	Male	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	54.0 +/- 18.0 uM	Adult (>18 years old)	Female	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	109.0 +/- 32.0 uM	Adult (>18 years old)	Both	Normal	16635166	details
Blood	Detected and Quantified	34.3 +/- 13.0 uM	Children (1-13 years old)	Both	Normal	11865086	details
Blood	Detected and Quantified	185 (144-222) uM	Newborn (0-30 days old)	Not Available	Normal	25754623	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Blood	Detected and Quantified	31-179 uM	Infant (0-1 year old)	Not Specified	Normal	9950309	details
Blood	Detected and Quantified	124 (90.4-189) uM	Infant (0-1 year old)	Not Available	Normal	25754623	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	22892715	details
Blood	Detected and Quantified	30-64 uM	Adult (>18 years old)	Not Specified	Normal	4122112	details
Blood	Detected and Quantified	72 +/- 25 uM	Children (1-13 years old)	Not Specified	Normal	11092761	details
Blood	Detected and Quantified	20-135 uM	Children (1-13 years old)	Not Specified	Normal	25691415	details
Blood	Detected and Quantified	20-135 uM	Infant (0-1 year old)	Not Specified	Normal	25691415	details
Blood	Detected and Quantified	54 +/- 18 uM	Adult (>18 years old)	Female	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	30.00-110.0 uM	Adult (>18 years old)	Both	Normal	Molecular You	details
Blood	Detected and Quantified	69.0 +/- 18.0 uM	Adult (>18 years old)	Both	Normal	28278231	details
Blood	Detected and Quantified	65 +/- 18 uM	Adult (>18 years old)	Male	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	69.1(56.1-90.2) uM	Children (1-13 uears old)	Both	Normal	27294788	details
Blood	Detected and Quantified	10-110 uM	Children (1-13 years old)	Both	Normal	2044610	details
Blood	Detected and Quantified	48-211 uM	Infant (0-1 year old)	Not Specified	Normal	20549362	details
Blood	Detected and Quantified	74.3 (52.7-81.3) uM	Adult (>18 years old)	Both	Normal	27506743	details
Blood	Detected and Quantified	77 +/- 41 uM	Infant (0-1 year old)	Both	Normal	7623444	details
Blood	Detected and Quantified	8-156 uM	Children (1 - 13 years old)	Both	Normal	28028301	details
Blood	Detected and Quantified	38-207 uM	Newborn (0-30 days old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	26-117 uM	Infant (1 - 3 months old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	27-100 uM	Children (3 months - 6 years old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	19-100 uM	Children (6 - 18 years old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	66.9 +/- 15.3 uM	Adult (>18 years old)	Both	Normal	21359215	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	4.87 (3.48-6.26) uM	Adult (>18 years old)	Both	Normal	12834252	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	8.3 +/- 4.7 uM	Adult (>18 years old)	Not Specified	Normal	15911239	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	6.0 +/- 1.5 uM	Adult (>18 years old)	Not Specified	Normal	6198473	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	4.9 +/- 1.6 uM	Adult (>18 years old)	Not Specified	Normal	6198473	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	3.7 +/- 1.0 uM	Adult (>18 years old)	Both	Normal	Geigy Scientific ...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	8.1(5-7-10-9) uM	Adult (>18 years old)	Not Specified	Normal	4122112	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	2.4-9.0 uM	Children (1-13 years old)	Both	Normal	23890588	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	4.5 +/- 2.2 uM	Adult (>18 years old)	Not Specified	Normal	22546835	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	2.3-9.5 uM	Children (0 - 10 years old)	Both	Normal	BC Children's Hos...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	2.3-11.2 uM	Adolescent (>11 years old)	Both	Normal	BC Children's Hos...	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	29808030	details
Feces	Detected but not Quantified	Not Quantified	Infant (0-1 year old)	Both	Normal	22411374	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Both	Normal	24130822	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Both	Normal	25486321	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Normal	25985090	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27543620	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	25037050	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Both	Normal	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Normal	27609529	details
Feces	Detected and Quantified	20 +/- 20 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	20669995	details
Saliva	Detected and Quantified	12.5 +/- 10.8 uM	Adult (>18 years old)	Not Specified	Normal	Physiological and...	details
Saliva	Detected and Quantified	14.2 +/- 11.1 uM	Adult (>18 years old)	Female	Normal	Physiological and...	details
Saliva	Detected and Quantified	16.7 +/- 14.6 uM	Adult (>18 years old)	Not Specified	Normal	Physiological and...	details
Saliva	Detected and Quantified	18.3 +/- 14.2 uM	Adult (>18 years old)	Not Specified	Normal	Physiological and...	details
Saliva	Detected and Quantified	28.9 +/- 33.1 uM	Adult (>18 years old)	Female	Normal	Physiological and...	details
Saliva	Detected and Quantified	28.9 +/- 33.1 uM	Adult (>18 years old)	Not Specified	Normal	Physiological and...	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	20300169	details
Saliva	Detected and Quantified	13.4 +/- 7.5 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	11.5 +/- 6.3 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	14.0 +/- 10.6 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	65.34 +/- 15.64 uM	Adult (>18 years old)	Both	Normal	Zerihun T. Dame, ...	details
Saliva	Detected and Quantified	18.5 +/- 8.6 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	>10 uM	Adult (>18 years old)	Both	Normal	12097436	details
Saliva	Detected and Quantified	38.16 +/- 52.83 uM	Adult (>18 years old)	Female	Normal	23857558	details
Sweat	Detected and Quantified	< 10 uM	Adult (60 years old)	Male	Normal	22194922	details
Sweat	Detected and Quantified	< 10 uM	Adult (40 years old)	Male	Normal	22194922	details
Sweat	Detected but not Quantified	Not Quantified	Adult	Both	Normal	26755145	details
Urine	Detected and Quantified	0-5 umol/mmol creatinine	Children (1-13 years old)	Both	Normal	23890588	details
Urine	Detected and Quantified	5.3 (2.0-8.8) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	<1.92 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	1.44-24.36 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	López Hernández Y...	details
Urine	Detected and Quantified	4.72(2.03-10.45) umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	López Hernández Y...	details
Urine	Detected and Quantified	5.79(1.28-25.46) umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	López Hernández Y...	details
Urine	Detected and Quantified	0.98 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	19309105	details
Urine	Detected and Quantified	<2.036 umol/mmol creatinine	Adolescent (13-18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21389975	details
Urine	Detected and Quantified	12.584 +/- 12.605 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Mordechai, Hien, ...	details
Urine	Detected and Quantified	0 - 24.08 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	2.19 +/- 3.21 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	2026685	details
Urine	Detected and Quantified	0 - 13.00 umol/mmol creatinine	Infant (1 - 6 months old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 10.40 umol/mmol creatinine	Infant (6 months - <1 year old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 10.40 umol/mmol creatinine	Children (1 - 2 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 9.04 umol/mmol creatinine	Children (2 - 4 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 9.04 umol/mmol creatinine	Children (4 - 13 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 6.56 umol/mmol creatinine	Adolescent (13 - 21 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 6.56 umol/mmol creatinine	Adult (>21 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	1.5-2.6 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	27012787	details
Urine	Detected and Quantified	<2.83 umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	2.733 +/- 1.313 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	Geigy Scientific ...	details
Urine	Detected and Quantified	2.0 (0.90-2.94) umol/mmol creatinine	Adult (>18 years old)	Male	Normal	5472370	details
Urine	Detected and Quantified	3.147 +/- 1.487 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	Geigy Scientific ...	details
Urine	Detected and Quantified	<29.97 umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	0.0024 - 0.0111 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	21389975	details
Urine	Detected and Quantified	0.787 +/- 0.373 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Geigy Scientific ...	details
Urine	Detected and Quantified	0.593 +/- 0.340 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Geigy Scientific ...	details
Urine	Detected and Quantified	0.987 (0.132-1.842) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	Not Available	details
Urine	Detected and Quantified	1.882 +/- 1.968 umol/mmol creatinine	Not Specified	Not Specified	Normal	5727921	details
Urine	Detected and Quantified	4.11 +/- 1.37 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	15992788	details
Urine	Detected and Quantified	4.4 (1.2-22.1) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	3.34 (0-6.67) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	0.78 +/- 0.36 umol/mmol creatinine	Children (1-13 years old)	Male	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	<7.92 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	3.1 +/- 1.46 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	2.6 +/- 1.30 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	0-7 umol/mmol creatinine	Children (1 - 13 years old)	Both	Normal	28028301	details
Urine	Detected and Quantified	1.696-46.468 umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	20549362	details
Urine	Detected and Quantified	1.882 +/- 1.968 umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	5727921	details
Urine	Detected and Quantified	1.2-2.3 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	27012787	details
Urine	Detected and Quantified	0-17 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	30830347	details
Urine	Detected and Quantified	0.9 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	21388201	details
Urine	Detected and Quantified	2.471 +/- 0.8319 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Mordechai, Hien, ...	details
Urine	Detected and Quantified	<4.98 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	31.66 +/- 29.40 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	54.27 +/- 47.49 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	76.88 +/- 79.14 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	30.5 (12.1) uM	Adult (>18 years old)	Female	Down syndrome pregnancy	23313728	details
Blood	Detected and Quantified	55.2 +/- 15.4 uM	Children (1-13 years old)	Both	Obesity	Metabolomics reve...	details
Blood	Detected and Quantified	26.0 (10.8) uM	Adult (>18 years old)	Female	Pregnancy	23313728	details
Blood	Detected and Quantified	60.2 +/- 18.2 uM	Children (1-13 years old)	Both	Obesity	Metabolomics reve...	details
Blood	Detected and Quantified	65.4 +/- 30.4 uM	Adult (>18 years old)	Both	Heart Transplant	21359215	details
Blood	Detected and Quantified	21 (2-83) uM	Adult (>18 years old)	Not Specified	Lysinuric protein intolerance	Lysinuric Protein...	details
Blood	Detected and Quantified	41.0 +/- 16.0 uM	Adult (>18 years old)	Both	Ornithine transcarbamylase (OTC) deficiency	16635166	details
Blood	Detected and Quantified	36.83 +/- 13.32 uM	Adult (>18 years old)	Female	Pregnancy with fetuses with trisomy 18	23535240	details
Blood	Detected and Quantified	28.20 +/- 10.61 uM	Adult (>18 years old)	Female	Pregnancy	23535240	details
Blood	Detected and Quantified	37.8 +/- 12.0 uM	Children (1-13 years old)	Both	Uremia	11865086	details
Blood	Detected and Quantified	36.1 (11.9) uM	Adult (>18 years old)	Female	Early preeclampsia	22494326	details
Blood	Detected and Quantified	38.2 (15.7) uM	Adult (>18 years old)	Female	Pregnancy	22494326	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25105552	details
Blood	Detected and Quantified	191 uM	Newborn (0-30 days old)	Male	Narp Syndrome	9950309	details
Blood	Detected and Quantified	23-54 uM	Children (1-13 years old)	Male	Narp Syndrome	9950309	details
Blood	Detected and Quantified	20-153 uM	Infant (0-1 year old)	Both	Narp Syndrome	9950309	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Schizophrenia	22892715	details
Blood	Detected and Quantified	25.15 (7.57) uM	Adult (>18 years old)	Female	Pregnancy with fetus having congenital heart defect	24704061	details
Blood	Detected and Quantified	22.37 (8.47) uM	Adult (>18 years old)	Female	Pregnancy	24704061	details
Blood	Detected and Quantified	33 +/- 29 (9-98) uM	Children (1-13 years old)	Female	Pearson Syndrome	25691415	details
Blood	Detected and Quantified	1066(798-1321) uM	Adult (>18 years old)	Both	Hyperornithinemia with gyrate atrophy	4122112	details
Blood	Detected and Quantified	33 +/- 15 uM	Children (1-13 years old)	Both	Cutis laxa, autosomal recessive, type IIIA	11092761	details
Blood	Detected and Quantified	19 uM	Infant (0-1 year old)	Female	Pearson Syndrome	25691415	details
Blood	Detected and Quantified	36.8 (17.4) uM	Adult (>18 years old)	Female	Late-onset preeclampsia	23159745	details
Blood	Detected and Quantified	42.3 (22.5) uM	Adult (>18 years old)	Female	Pregnancy	23159745	details
Blood	Detected and Quantified	74.9(54.3-116.5) uM	Children (1-13 uears old)	Both	Environmental enteric dysfunction	27294788	details
Blood	Detected and Quantified	68.35 +/- 22.43 uM	Adult (>18 years old)	Both	Alzheimer's disease	17031479	details
Blood	Detected and Quantified	77 uM	Infant (0-1 year old)	Male	Fumaric aciduria	20549362	details
Blood	Detected and Quantified	32-167 uM	Newborn (0-30 days old)	Male	N-Acetylglutamate synthetase deficiency	7623444	details
Blood	Detected and Quantified	32.3 (22.4-53.9) uM	Adult (>18 years old)	Both	Lysinuric protein intolerance	27506743	details
Blood	Detected and Quantified	4-22 uM	Children (1 - 13 years old)	Both	Lysinuric protein intolerance	28028301	details
Blood	Detected and Quantified	42-290 uM	Children (1-13 years old)	Male	N-acetylglutamate synthetase deficiency	2044610	details
Blood	Detected and Quantified	53-152 uM	Infant (0-1 year old)	Male	N-Acetylglutamate synthetase deficiency	7623444	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	7.6 +/- 3.1 uM	Children (1-13 years old)	Not Specified	Leukemia	15911239	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	6.9 +/- 3.6 uM	Children (1-13 years old)	Not Specified	Leukemia	15911239	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	288(240-314) uM	Adult (>18 years old)	Both	Hyperornithinemia with gyrate atrophy	4122112	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25037050	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Both	Autism	24130822	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Both	Crohns disease	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Both	Ulcerative colitis	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Crohns disease	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Ulcerative colitis	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Unclassified IBD	27609529	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	27015276	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Oral cancer	20300169	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Breast cancer	20300169	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Pancreatic cancer	20300169	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Periodontal diseases	20300169	details
Saliva	Detected and Quantified	28.26 +/- 9.78 uM	Adult (>18 years old)	Male	Alzheimer's disease	23857558	details
Saliva	Detected and Quantified	24.28 +/- 12.91 uM	Adult (>18 years old)	Male	Frontotemporal lobe dementia	23857558	details
Saliva	Detected and Quantified	16.85 +/- 10.62 uM	Adult (>18 years old)	Both	Lewy body disease	23857558	details
Urine	Detected and Quantified	26-63 umol/mmol creatinine	Children (1-13 years old)	Both	Hyperlysinemia I, familial	23890588	details
Urine	Detected and Quantified	19-988.300 umol/mmol creatinine	Adult (>18 years old)	Both	Cystinuria	14586528	details
Urine	Detected and Quantified	11.611 +/- 17.647 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Mordechai, Hien, ...	details
Urine	Detected and Quantified	5.0 (2.0-8.0) umol/mmol creatinine	Adult (>18 years old)	Both	Cystinuria	MetaGene: Metabol...	details
Urine	Detected and Quantified	350.0 (200.0-500.0) umol/mmol creatinine	Children (1-13 years old)	Both	Cystinuria	MetaGene: Metabol...	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Bladder cancer	28157703	details
Urine	Detected and Quantified	4.363-53.381 umol/mmol creatinine	Children (1-13 years old)	Female	Dibasic Amino Aciduria I	5727921	details
Urine	Detected and Quantified	4.106-35.759 umol/mmol creatinine	Adult (>18 years old)	Both	Dibasic Amino Aciduria I	5727921	details
Urine	Detected and Quantified	12.34 +/- 5.07 umol/mmol creatinine	Adult (>18 years old)	Both	Alzheimer's disease	17031479	details
Urine	Detected and Quantified	190-9883 umol/mmol creatinine	Adult (>18 years old)	Both	Hyperlysinuria	14586528	details
Urine	Detected and Quantified	16.110-51 umol/mmol creatinine	Children (1 - 13 years old)	Both	Lysinuric protein intolerance	28028301	details
Urine	Detected and Quantified	332.7 umol/mmol creatinine	Adult (>18 years old)	Both	Cystinuria	14586528	details
Urine	Detected and Quantified	4.106-13.859 umol/mmol creatinine	Adult (>18 years old)	Both	Hyperdibasic aminoaciduria I	5727921	details
Urine	Detected and Quantified	8.897-14.0297 umol/mmol creatinine	Children (1 - 13 years old)	Female	Hyperdibasic aminoaciduria I	5727921	details
Urine	Detected and Quantified	92.570-123.800 umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Fumaric aciduria	20549362	details
Urine	Detected and Quantified	3.371 +/- 6.3791 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Mordechai, Hien, ...	details
Urine	Detected and Quantified	2.8009 +/- 3.4795 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Gastroesophageal reflux disease	Mordechai, Hien, ...	details

Associated Disorders and Diseases

Disease References

Alzheimer's disease
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 ] 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
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
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
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 ] 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 ] 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
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
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
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 ] 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 ] 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 ] 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 ] 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
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
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 ] 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 ] 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
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
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 ] 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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Eosinophilic esophagitis
(). Mordechai, Hien, and David S. Wishart. .
Hyperdibasic aminoaciduria I
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
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
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

104300 (Alzheimer's disease)
311250 (Ornithine transcarbamylase deficiency)
114500 (Colorectal cancer)
181500 (Schizophrenia)
222700 (Lysinuric protein intolerance)
606812 (Fumarase deficiency)
608300 (N-acetylglutamate synthetase deficiency)
601665 (Obesity)
557000 (Pearson Syndrome)
258870 (Hyperornithinemia with Gyrate Atrophy)
219150 (Cutis laxa, autosomal recessive, type IIIA)
600462 (Myopathy, lactic acidosis, and sideroblastic anemia 1)
209850 (Autism)
266600 (Crohn's disease)
260350 (Pancreatic cancer)
170650 (Periodontal disease)
600274 (Frontotemporal dementia)
220100 (Cystinuria)
610247 (Eosinophilic esophagitis)
222690 (Hyperdibasic aminoaciduria I)
238700 (Hyperlysinemia I, familial)
247950 (Hyperlysinuria)

External Links

DrugBank ID

DB00129

Phenol Explorer Compound ID

Not Available

FooDB ID

KNApSAcK ID

Chemspider ID

KEGG Compound ID

BioCyc ID

BiGG ID

Wikipedia Link

METLIN ID

PubChem Compound

6262

PDB ID

Not Available

ChEBI ID

15729

Food Biomarker Ontology

Not Available

VMH ID

ORN

MarkerDB ID

MDB00000102

References

Synthesis Reference

Zhang, 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

Mayer UM: [Hyperornithinaemia in patients with retinal dystrophy]. Ophthalmologe. 2003 Jan;100(1):55-61. [PubMed:12557027 ]
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 ]
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 ]
Gray RG, Green A, Hall S, McKeown C: Prenatal exclusion of the HHH syndrome. Prenat Diagn. 1995 May;15(5):474-6. [PubMed:7644438 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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 ]
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.

Show all enzymes and transporters

Enzymes

Enzyme Details

1. Arginase-1

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 + Urea	details

References

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 ]
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 ]
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 ]
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 ]
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 ]

Enzyme Details

2. Ornithine aminotransferase, mitochondrial

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 acid	details
Ornithine + Oxoglutaric acid → L-Glutamic gamma-semialdehyde + L-Glutamic acid	details

References

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 ]
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 ]
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 ]

Enzyme Details

3. Glycine amidinotransferase, mitochondrial

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 acid	details
L-Arginine + gamma-Aminobutyric acid → Ornithine + 4-Guanidinobutanoic acid	details

References

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 ]
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 ]
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 ]
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 ]
Mitruka BM, Costilow RN: Arginine and ornithine catabolism by Clostridium botulinum. J Bacteriol. 1967 Jan;93(1):295-301. [PubMed:5335895 ]

Enzyme Details

4. Aminoacylase-1

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 + Ornithine	details

Enzyme Details

5. Delta-1-pyrroline-5-carboxylate synthase

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

Enzyme Details

6. Ornithine carbamoyltransferase, mitochondrial

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 + Citrulline	details

References

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 ]
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 ]
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 ]
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 ]

Enzyme Details

7. Ornithine decarboxylase

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 dioxide	details

Enzyme Details

8. Arginase-2, mitochondrial

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 + Urea	details

References

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 ]
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 ]
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 ]
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 ]
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 ]

Enzyme Details

9. Cationic amino acid transporter 4

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

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 ]
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 ]

Enzyme Details

10. Mitochondrial ornithine transporter 1

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

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 ]
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.

Show all enzymes and transporters

Showing metabocard for Ornithine (HMDB0000214)

MOL for HMDB0000214 (Ornithine)

3D MOL for HMDB0000214 (Ornithine)

3D SDF for HMDB0000214 (Ornithine)

3D-SDF for HMDB0000214 (Ornithine)

PDB for HMDB0000214 (Ornithine)

3D PDB for HMDB0000214 (Ornithine)

SMILES for HMDB0000214 (Ornithine)

INCHI for HMDB0000214 (Ornithine)

Structure for HMDB0000214 (Ornithine)

3D Structure for HMDB0000214 (Ornithine)

Collision Cross Sections

Retention Indices

Underivatized

Derivatized

GC-MS

LC-MS/MS

NMR

Enzymes

Reactions

References

Reactions

References

Reactions

References

Reactions

Reactions

References

Reactions

Reactions

References

References

References