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Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (29)transporters (1) Show 30 proteins XML

enzymes (29)transporters (1) Show 30 proteins

Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2023-07-07 20:54:00 UTC

HMDB ID

HMDB0000162

Secondary Accession Numbers

HMDB00162

Metabolite Identification

Common Name

Proline

Description

Proline (Pro), also known as L-proline is an alpha-amino acid. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon). Amino acids are organic compounds that contain amino (-NH2) and carboxyl (-COOH) functional groups, along with a side chain (R group) specific to each amino acid. Proline is one of 20 proteinogenic amino acids, i.e., the amino acids used in the biosynthesis of proteins. Proline is found in all organisms ranging from bacteria to plants to animals. It is classified as an aliphatic, non-polar amino acid. Proline is sometimes called an imino acid, although the IUPAC definition of an imine requires a carbon-nitrogen double bond. Proline is a non-essential amino acid that is synthesized from glutamic acid. It is an essential component of collagen and is important for proper functioning of joints and tendons. Proline is derived from the amino acid L-glutamate in which glutamate-5-semialdehyde is first formed by glutamate 5-kinase and glutamate-5-semialdehyde dehydrogenase (which requires NADH or NADPH). This semialdehyde can then either spontaneously cyclize to form 1-pyrroline-5-carboxylic acid, which is reduced to proline by pyrroline-5-carboxylate reductase, or turned into ornithine by ornithine aminotransferase, followed by cyclization by ornithine cyclodeaminase to form proline. L-Proline has been found to act as a weak agonist of the glycine receptor and of both NMDA and non-NMDA ionotropic glutamate receptors. It has been proposed to be a potential endogenous excitotoxin/neurotoxin. Studies in rats have shown that when injected into the brain, proline non-selectively destroys pyramidal and granule cells (PMID: 3409032 ). Therefore, under certain conditions proline can act as a neurotoxin and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of proline are associated with at least five inborn errors of metabolism, including hyperprolinemia type I, hyperprolinemia type II, iminoglycinuria, prolinemia type II, and pyruvate carboxylase deficiency. People with hyperprolinemia type I often do not show any symptoms even though they have proline levels in their blood between 3 and 10 times the normal level. Some individuals with hyperprolinemia type I exhibit seizures, intellectual disability, or other neurological or psychiatric problems. Hyperprolinemia type II results in proline levels in the blood between 10 and 15 times higher than normal, and high levels of a related compound called pyrroline-5-carboxylate. Hyperprolinemia type II has signs and symptoms that vary in severity and is more likely than type I to involve seizures or intellectual disability.

Structure

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

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
(-)-(S)-Proline	ChEBI
(-)-2-Pyrrolidinecarboxylic acid	ChEBI
(-)-Proline	ChEBI
(2S)-Pyrrolidine-2-carboxylic acid	ChEBI
(S)-2-Carboxypyrrolidine	ChEBI
(S)-2-Pyrrolidinecarboxylic acid	ChEBI
(S)-Pyrrolidine-2-carboxylic acid	ChEBI
2-Pyrrolidinecarboxylic acid	ChEBI
L-(-)-Proline	ChEBI
L-alpha-Pyrrolidinecarboxylic acid	ChEBI
L-Prolin	ChEBI
L-Pyrrolidine-2-carboxylic acid	ChEBI
P	ChEBI
Prolina	ChEBI
PROLINE	ChEBI
Prolinum	ChEBI
(-)-2-Pyrrolidinecarboxylate	Generator
(2S)-Pyrrolidine-2-carboxylate	Generator
(S)-2-Pyrrolidinecarboxylate	Generator
(S)-Pyrrolidine-2-carboxylate	Generator
2-Pyrrolidinecarboxylate	Generator
L-a-Pyrrolidinecarboxylate	Generator
L-a-Pyrrolidinecarboxylic acid	Generator
L-alpha-Pyrrolidinecarboxylate	Generator
L-Α-pyrrolidinecarboxylate	Generator
L-Α-pyrrolidinecarboxylic acid	Generator
L-Pyrrolidine-2-carboxylate	Generator
(S)-(-)-Proline	HMDB
(S)-(-)-Pyrrolidine-2-carboxylate	HMDB
(S)-(-)-Pyrrolidine-2-carboxylic acid	HMDB
(S)-2-Pyrralidinecarboxylate	HMDB
(S)-2-Pyrralidinecarboxylic acid	HMDB
(S)-Proline	HMDB
L Proline	HMDB

Chemical Formula

C₅H₉NO₂

Average Molecular Weight

115.1305

Monoisotopic Molecular Weight

115.063328537

IUPAC Name

(2S)-pyrrolidine-2-carboxylic acid

Traditional Name

L-proline

CAS Registry Number

147-85-3

SMILES

OC(=O)[C@@H]1CCCN1

InChI Identifier

InChI=1S/C5H9NO2/c7-5(8)4-2-1-3-6-4/h4,6H,1-3H2,(H,7,8)/t4-/m0/s1

InChI Key

ONIBWKKTOPOVIA-BYPYZUCNSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as proline and derivatives. Proline and derivatives are compounds containing proline or a derivative thereof resulting from reaction of proline at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

Proline and derivatives

Alternative Parents

Substituents

Proline or derivatives
Alpha-amino acid
L-alpha-amino acid
Pyrrolidine carboxylic acid
Pyrrolidine carboxylic acid or derivatives
Pyrrolidine
Amino acid
Carboxylic acid
Secondary aliphatic amine
Monocarboxylic acid or derivatives
Secondary amine
Organoheterocyclic compound
Azacycle
Organic oxygen compound
Organooxygen compound
Organonitrogen compound
Amine
Organopnictogen compound
Organic oxide
Carbonyl group
Organic nitrogen compound
Hydrocarbon derivative
Aliphatic heteromonocyclic compound

Molecular Framework

Aliphatic heteromonocyclic compounds

External Descriptors

proteinogenic amino acid (CHEBI:17203 )
L-alpha-amino acid (CHEBI:17203 )
proline (CHEBI:17203 )
glutamine family amino acid (CHEBI:17203 )
Common amino acids (C00148 )

Ontology

Not Available

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	221 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	162 mg/mL	Not Available
LogP	-2.54	HANSCH,C ET AL. (1995)

Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct Type	Data Source	CCS Value (Å²)	Reference
[M-H]-	Baker	133.514	30932474
[M-H]-	MetCCS_test_neg	122.4	30932474
[M+H]+	Astarita_pos	118.3	30932474
[M+H]+	Baker	126.48	30932474
[M+H]+	McLean	126.242	30932474
[M+H]+	MetCCS_test_pos	125.089	30932474
[M-H]-	Not Available	122.4	http://allccs.zhulab.cn/database/detail?ID=AllCCS00000077
[M+H]+	Not Available	125.7	http://allccs.zhulab.cn/database/detail?ID=AllCCS00000077

Predicted Molecular Properties

Property	Value	Source
Water Solubility	365 g/L	ALOGPS
logP	-2.7	ALOGPS
logP	-2.6	ChemAxon
logS	0.5	ALOGPS
pKa (Strongest Acidic)	1.94	ChemAxon
pKa (Strongest Basic)	11.33	ChemAxon
Physiological Charge	0	ChemAxon
Hydrogen Acceptor Count	3	ChemAxon
Hydrogen Donor Count	2	ChemAxon
Polar Surface Area	49.33 Å²	ChemAxon
Rotatable Bond Count	1	ChemAxon
Refractivity	28.06 m³·mol⁻¹	ChemAxon
Polarizability	11.5 Å³	ChemAxon
Number of Rings	1	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Predicted Chromatographic Properties

Predicted Collision Cross Sections

Predictor	Adduct Type	CCS Value (Å²)	Reference
DarkChem	[M+H]+	124.076	31661259
DarkChem	[M-H]-	118.356	31661259
AllCCS	[M+H]+	125.08	32859911
AllCCS	[M-H]-	120.874	32859911
DeepCCS	[M+H]+	119.333	30932474
DeepCCS	[M-H]-	115.749	30932474
DeepCCS	[M-2H]-	153.162	30932474
DeepCCS	[M+Na]+	128.317	30932474
AllCCS	[M+H]+	125.1	32859911
AllCCS	[M+H-H2O]+	120.3	32859911
AllCCS	[M+NH4]+	129.5	32859911
AllCCS	[M+Na]+	130.8	32859911
AllCCS	[M-H]-	120.9	32859911
AllCCS	[M+Na-2H]-	123.4	32859911
AllCCS	[M+HCOO]-	126.2	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
L-Proline	OC(=O)[C@@H]1CCCN1	1823.6	Standard polar	33892256
L-Proline	OC(=O)[C@@H]1CCCN1	1083.3	Standard non polar	33892256
L-Proline	OC(=O)[C@@H]1CCCN1	1293.2	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
L-Proline,1TMS,isomer #1	C[Si](C)(C)OC(=O)[C@@H]1CCCN1	1178.4	Semi standard non polar	33892256
L-Proline,1TMS,isomer #2	C[Si](C)(C)N1CCC[C@H]1C(=O)O	1275.7	Semi standard non polar	33892256
L-Proline,2TMS,isomer #1	C[Si](C)(C)OC(=O)[C@@H]1CCCN1[Si](C)(C)C	1311.0	Semi standard non polar	33892256
L-Proline,2TMS,isomer #1	C[Si](C)(C)OC(=O)[C@@H]1CCCN1[Si](C)(C)C	1357.1	Standard non polar	33892256
L-Proline,2TMS,isomer #1	C[Si](C)(C)OC(=O)[C@@H]1CCCN1[Si](C)(C)C	1555.9	Standard polar	33892256
L-Proline,1TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)[C@@H]1CCCN1	1420.9	Semi standard non polar	33892256
L-Proline,1TBDMS,isomer #2	CC(C)(C)[Si](C)(C)N1CCC[C@H]1C(=O)O	1551.5	Semi standard non polar	33892256
L-Proline,2TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)[C@@H]1CCCN1[Si](C)(C)C(C)(C)C	1769.8	Semi standard non polar	33892256
L-Proline,2TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)[C@@H]1CCCN1[Si](C)(C)C(C)(C)C	1775.0	Standard non polar	33892256
L-Proline,2TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)[C@@H]1CCCN1[Si](C)(C)C(C)(C)C	1834.0	Standard polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental GC-MS	GC-MS Spectrum - Proline GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-0006-0910000000-a9127c0e370afb80259b	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-000f-0910000000-3195f21e625520d51316	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)	splash10-0006-0900000000-119af746c347969e8ae7	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-006x-8900000000-b31beabb1ed6235e7145	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline GC-MS (1 TMS)	splash10-00di-9100000000-c1c67bc521741ee891cb	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline GC-MS (2 TMS)	splash10-0006-1900000000-84e6fb318323fd0261b1	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline GC-MS (1 TMS)	splash10-00xs-8910000000-74b0c34ca5fd7c85f1d9	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline EI-B (Non-derivatized)	splash10-00fu-9000000000-716c3d01b301162863b6	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline EI-B (Non-derivatized)	splash10-0006-0900000000-0d7354201ed866c87b13	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline GC-EI-TOF (Non-derivatized)	splash10-0006-0910000000-a9127c0e370afb80259b	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline GC-EI-TOF (Non-derivatized)	splash10-000f-0910000000-3195f21e625520d51316	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline GC-EI-TOF (Non-derivatized)	splash10-0006-0900000000-119af746c347969e8ae7	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline GC-EI-QQ (Non-derivatized)	splash10-001i-1920000000-09006ee4e7a137d8a45a	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline GC-EI-TOF (Non-derivatized)	splash10-006x-8900000000-b31beabb1ed6235e7145	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline GC-MS (Non-derivatized)	splash10-0006-1900000000-84e6fb318323fd0261b1	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline GC-MS (Non-derivatized)	splash10-00xs-8910000000-74b0c34ca5fd7c85f1d9	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Proline GC-MS (Non-derivatized)	splash10-00di-9100000000-c1c67bc521741ee891cb	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Proline GC-MS (Non-derivatized) - 70eV, Positive	splash10-00fu-9000000000-d203e3dfb0701403a75b	2016-09-22	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Proline GC-MS (1 TMS) - 70eV, Positive	splash10-00di-9000000000-56471980a375d26046ca	2017-10-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Proline GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Proline GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Proline GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Proline GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline Quattro_QQQ 10V, Positive-QTOF (Annotated)	splash10-00xr-9600000000-ba51a901e786b1a4663e	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline Quattro_QQQ 25V, Positive-QTOF (Annotated)	splash10-00di-9000000000-f72e2f3812225708fc56	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline Quattro_QQQ 40V, Positive-QTOF (Annotated)	splash10-00di-9000000000-59887da6580f63bcc1c5	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline EI-B (HITACHI M-80) , Positive-QTOF	splash10-00fu-9000000000-c69dcaf7cb2d1e9d285e	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-014i-0930000000-984f9a23ebfe28f95017	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-014i-9000000000-f75575cac28a54aa923d	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-00di-9000000000-221cfb8c82f3df76eb51	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-014i-0900000000-e5741fd9870218d57caf	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-014i-0900000000-6bc9b4e775be37fa18d9	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-014i-9000000000-a8eb419b26655a6a1dab	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-00di-9000000000-873ee03b61d1ffcae70f	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Positive-QTOF	splash10-014i-0900000000-61d583960651669fb9e6	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative-QTOF	splash10-03di-0900000000-2c23774af5f6a0c217d5	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative-QTOF	splash10-03di-0900000000-d89c1e626ef24a79c89f	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative-QTOF	splash10-03di-1900000000-fc27094f2eb75e65b438	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positive-QTOF	splash10-014i-1900000000-3221c23504b8847abf8d	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positive-QTOF	splash10-00di-9100000000-8169367f5127c57fae12	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positive-QTOF	splash10-00di-9000000000-f704eb8402fcacfccd18	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positive-QTOF	splash10-00di-9000000000-ccd74eea36efab2cbab8	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positive-QTOF	splash10-00di-9000000000-9f214692cd39a6581813	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline CE-ESI-TOF (CE-system connected to 6210 Time-of-Flight MS, Agilent) , Positive-QTOF	splash10-014i-0900000000-ef038c77922e4c89eeb0	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positive-QTOF	splash10-00di-9400000000-4073ea1df57a60b36d69	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) 30V, Positive-QTOF	splash10-00di-9000000000-495fdbd2f565bd794632	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Proline LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative-QTOF	splash10-03di-0900000000-e1d0a4b20419dc7bae92	2012-08-31	HMDB team, MONA	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Proline 10V, Positive-QTOF	splash10-014i-6900000000-eba8ceb2a71d2726acd0	2016-09-12	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Experimental 1D NMR	¹H NMR Spectrum (1D, 600 MHz, H₂O, experimental)	2012-12-04	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Experimental 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, H₂O, experimental)	2021-10-10	Wishart Lab	View Spectrum
Experimental 2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, H₂O, experimental)	2012-12-05	Wishart Lab	View Spectrum

IR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)	2023-02-03	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)	2023-02-03	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)	2023-02-03	FELIX lab	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm
Extracellular
Mitochondria
Lysosome
Endoplasmic reticulum

Biospecimen Locations

Blood
Breast Milk
Cerebrospinal Fluid (CSF)
Feces
Saliva
Sweat
Urine

Tissue Locations

All Tissues
Placenta
Prostate

Pathways

Name	SMPDB/PathBank	KEGG
Transcription/Translation	SMP00019	Not Available
Arginine and proline metabolism	Not Available
Prolidase Deficiency (PD)		Not Available
Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency)		Not Available
Hyperprolinemia Type II		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	198.3 +/- 64.8 uM	Adult (>18 years old)	Both	Normal	21359215	details
Blood	Detected and Quantified	168.0 (108.0-228.0) uM	Adult (>18 years old)	Both	Normal	12297216	details
Blood	Detected and Quantified	177.5 +/- 38.6 uM	Adult (>18 years old)	Both	Normal	21359215	details
Blood	Detected and Quantified	111-329 uM	Newborn (0-30 days old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	77-324 uM	Infant (1 - 3 months old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	40-332 uM	Children (3 months - 6 years old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	58-324 uM	Children (6 - 18 years old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	198.0 +/- 63.0 uM	Children (1-13 years old)	Male	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	239.0 +/- 70.0 uM	Adult (>18 years old)	Male	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	168.0 +/- 49.0 uM	Adult (>18 years old)	Female	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	61-285 uM	Infant (0-1 year old)	Not Specified	Normal	24341803	details
Blood	Detected and Quantified	230.0 +/- 75.0 uM	Newborn (0-30 days old)	Not Specified	Normal	Geigy Scientific ...	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Blood	Detected and Quantified	211 (184-247) uM	Newborn (0-30 days old)	Not Available	Normal	25754623	details
Blood	Detected and Quantified	180 +/- 47 uM	Children (1-13 years old)	Not Specified	Normal	11092761	details
Blood	Detected and Quantified	51-271 uM	Children (1-13 years old)	Not Specified	Normal	24931297	details
Blood	Detected and Quantified	93-233 uM	Children (1-13 years old)	Not Specified	Normal	28649521	details
Blood	Detected and Quantified	222 (159-278) uM	Infant (0-1 year old)	Not Specified	Normal	894411	details
Blood	Detected and Quantified	193 +/- 52 uM	Infant (0-1 year old)	Both	Normal	432003	details
Blood	Detected and Quantified	256 (207-310) uM	Infant (0-1 year old)	Not Available	Normal	25754623	details
Blood	Detected and Quantified	178 +/- 33 uM	Children (1 - 13 years old)	Both	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	105.0-360.0 uM	Adult (>18 years old)	Both	Normal	Molecular You	details
Blood	Detected and Quantified	188.3 +/- 55.0 uM	Adult (>18 years old)	Both	Normal	28278231	details
Blood	Detected and Quantified	179.7(156.2-221.1) uM	Children (1-13 uears old)	Both	Normal	27294788	details
Blood	Detected and Quantified	258.91 +/- 91 uM	Children (1-13 years old)	Both	Normal	23108202	details
Breast Milk	Detected and Quantified	27.2 +/- 20.0 uM	Adult (>18 years old)	Female	Normal	24027187	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	4.0 (0 - 8.0) uM	Adult (>18 years old)	Both	Normal	11510941	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.6 uM	Infant (0-1 year old)	Not Specified	Normal	894411	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	1.9 +/- 1.0 uM	Adult (>18 years old)	Not Specified	Normal	22546835	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0-3 uM	Children (0 - 10 years old)	Both	Normal	BC Children's Hos...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0-3 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	25670064	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	23940645	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Both	Normal	24130822	details
Feces	Detected but not Quantified	Not Quantified	Infant (0-1 year old)	Both	Normal	22411374	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Both	Normal	25486321	details
Feces	Detected and Quantified	9641.233 (5153.572-14128.894) nmol/g wet feces	Infant (0-1 year old)	Not Specified	Normal	22435727	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Both	Normal	21970810	details
Feces	Detected and Quantified	15258.0478 (7151.305-23364.790) nmol/g wet feces	Infant (0-1 year old)	Not Specified	Normal	22435727	details
Feces	Detected and Quantified	20643.241 (12681.262-28605.220) nmol/g wet feces	Infant (0-1 year old)	Not Specified	Normal	22435727	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Normal	27609529	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	18052033	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	19573517	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21761941	details
Feces	Detected and Quantified	19.109 +/- 10.0755 nmol/g wet feces	Not Specified	Not Specified	Normal	19573517	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)	Female	Normal	26709303	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27065191	details
Feces	Detected and Quantified	950.226 +/- 727.870 nmol/g wet feces	Not Specified	Not Specified	Normal	20669995	details
Feces	Detected and Quantified	510 +/- 430 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	19006102	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Not Specified	Normal	27452636	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	25037050	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	28587349	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27107423	details
Feces	Detected and Quantified	180 +/- 160 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Not Specified	Normal	21970810	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	17269711	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	29808030	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	20669995	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	20300169	details
Saliva	Detected and Quantified	118 +/- 125 uM	Adult (>18 years old)	Male	Normal	Sugimoto et al. (...	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21190195	details
Saliva	Detected and Quantified	18.8 +/- 13.5 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected and Quantified	11.4 +/- 9.9 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	6.1 +/- 5.3 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Normal	22727327	details
Saliva	Detected and Quantified	6.0 +/- 2.6 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	158.48 +/- 122.10 uM	Adult (>18 years old)	Both	Normal	Zerihun T. Dame, ...	details
Saliva	Detected and Quantified	9.5 +/- 10.5 uM	Adult (>18 years old)	Male	Normal	21164041	details
Saliva	Detected and Quantified	96.85 +/- 74.83 uM	Adult (>18 years old)	Female	Normal	23857558	details
Saliva	Detected and Quantified	>10 uM	Adult (>18 years old)	Both	Normal	12097436	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
Urine	Detected and Quantified	<2.26 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	0.86 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	19309105	details
Urine	Detected and Quantified	<3.17 umol/mmol creatinine	Adolescent (13-18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	39.537 +/- 19.358 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details
Urine	Detected and Quantified	<6.08-34.76 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	David F. Putnam C...	details
Urine	Detected and Quantified	0.39-0.85 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	27012787	details
Urine	Detected and Quantified	<2.94 umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	3.17-229.48 umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	0 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	19033659	details
Urine	Detected and Quantified	0 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	19033659	details
Urine	Detected and Quantified	<2.0 umol/mmol creatinine	Infant (6 months - <1 year old)	Both	Normal	3168222	details
Urine	Detected and Quantified	0 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	19033659	details
Urine	Detected and Quantified	<9.0 umol/mmol creatinine	Infant (1 - 6 months old)	Both	Normal	3168222	details
Urine	Detected and Quantified	3.6 +/- 1.4 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	3168222	details
Urine	Detected and Quantified	0-3 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	19033659	details
Urine	Detected and Quantified	3.06 /- 1.87 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	15992788	details
Urine	Detected and Quantified	2.171 (0.0658-4.276) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	Not Available	details
Urine	Detected and Quantified	1.1 (0.1-2.5) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	<13.46 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	84.89 (34.67-135.11) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Geigy Scientific ... West Cadwell, N.J... Basel, Switzerlan...	details
Urine	Detected and Quantified	21.93 - 220.92 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 134.88 umol/mmol creatinine	Infant (1 - 6 months old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 14.70 umol/mmol creatinine	Infant (6 months - <1 year old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 13.57 umol/mmol creatinine	Children (1 - 2 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 9.50 umol/mmol creatinine	Children (2 - 4 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 9.50 umol/mmol creatinine	Children (4 - 13 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 9.50 umol/mmol creatinine	Adolescent (13 - 21 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 9.50 umol/mmol creatinine	Adult (>21 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	8-260 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	30830347	details
Urine	Detected and Quantified	8-260 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	30830347	details
Urine	Detected and Quantified	0.41-0.66 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	27012787	details
Urine	Detected and Quantified	248.73 +/- 180.90 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	47.98 (34.13-109.22) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Analysis of 40 NI...	details
Urine	Detected and Quantified	440.93 +/- 260.04 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	508.77 +/- 237.43 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	19468821	details
Urine	Detected and Quantified	4.14 +/- 2.85 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	2026685	details
Urine	Detected and Quantified	0.5 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	21388201	details
Urine	Detected and Quantified	<8.82 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	Mayo Clinic	details
Urine	Detected and Quantified	2.4998 +/- 0.8786 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details
Urine	Detected and Quantified	9.5-78.2 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	32340350	details
Urine	Detected and Quantified	22.36(12.65-53.64) umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	32340350	details
Urine	Detected and Quantified	23(9.49-82.39) umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	32340350	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	164.6 +/- 49.5 uM	Children (1-13 years old)	Both	Obesity	Metabolomics reve...	details
Blood	Detected and Quantified	197.2 +/- 71.5 uM	Children (1-13 years old)	Both	Obesity	Metabolomics reve...	details
Blood	Detected and Quantified	159.9 +/- 86.3 uM	Adult (>18 years old)	Both	Heart Transplant	21359215	details
Blood	Detected and Quantified	208.459-1745.845 uM	Children (1-13 years old)	Both	Hyperprolinemia, type I	14497974	details
Blood	Detected and Quantified	135.31 +/- 42.98 uM	Adult (>18 years old)	Female	Pregnancy with fetuses with trisomy 18	23535240	details
Blood	Detected and Quantified	125.42 +/- 48.53 uM	Adult (>18 years old)	Female	Pregnancy	23535240	details
Blood	Detected and Quantified	138.6 (52.0) uM	Adult (>18 years old)	Female	Early preeclampsia	22494326	details
Blood	Detected and Quantified	142.6 (58.5) uM	Adult (>18 years old)	Female	Pregnancy	22494326	details
Blood	Detected and Quantified	417.5 (179-576) uM	Children (1-13 years old)	Male	Lipoyltransferase 1 Deficiency	24341803	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25105552	details
Blood	Detected and Quantified	201.0 +/- 149.5 uM	Adult (>18 years old)	Both	Hemodialysis	10838467	details
Blood	Detected and Quantified	227.2 +/- 108.4 uM	Adult (>18 years old)	Both	Hemodialysis	10838467	details
Blood	Detected and Quantified	390.861 uM	Infant (0-1 year old)	Male	Dihydrolipoamide Dehydrogenase Deficiency	6688766	details
Blood	Detected and Quantified	>1500 uM	Children (1-13 years old)	Not Specified	Hyperprolinemia, type II	24931297	details
Blood	Detected and Quantified	801 uM	Infant (0-1 year old)	Not Available	Pyruvate carboxylase deficiency	28649521	details
Blood	Detected and Quantified	91 +/- 18 uM	Children (1-13 years old)	Both	Cutis laxa, autosomal recessive, type IIIA	11092761	details
Blood	Detected and Quantified	212 (70-388) uM	Infant (0-1 year old)	Male	Dicarboxylic Aminoaciduria	894411	details
Blood	Detected and Quantified	149-233 uM	Newborn (0-30 days old)	Female	Mitochondrial pyruvate carrier deficiency	12649063	details
Blood	Detected and Quantified	114 uM	Children (1-13 years old)	Not Available	Pyruvate carboxylase deficiency	28649521	details
Blood	Detected and Quantified	159 +/- 47 uM	Children (1-13 years old)	Both	Obese	24740590	details
Blood	Detected and Quantified	166 +/- 46 uM	Children (1-13 years old)	Both	Obese	24740590	details
Blood	Detected and Quantified	154 +/- 48 uM	Children (1-13 years old)	Both	Obese	24740590	details
Blood	Detected and Quantified	163 +/- 47 uM	Children (1-13 years old)	Both	Obese	24740590	details
Blood	Detected and Quantified	111.43 (37.73) uM	Adult (>18 years old)	Female	Pregnancy with fetus having congenital heart defect	24704061	details
Blood	Detected and Quantified	106 (33.59) uM	Adult (>18 years old)	Female	Pregnancy	24704061	details
Blood	Detected and Quantified	650.00 (300.00-1000.00) uM	Children (1-13 years old)	Both	Glutathione synthetase deficiency	MetaGene: Metabol...	details
Blood	Detected and Quantified	108-533 uM	Infant (0-1 year old)	Both	Iminoglycinuria	432003	details
Blood	Detected and Quantified	172.2 (57.7) uM	Adult (>18 years old)	Female	Late-onset preeclampsia	23159745	details
Blood	Detected and Quantified	165.7 (56.4) uM	Adult (>18 years old)	Female	Pregnancy	23159745	details
Blood	Detected and Quantified	191.5(163.3-226.4) uM	Children (1-13 uears old)	Both	Environmental enteric dysfunction	27294788	details
Blood	Detected and Quantified	180.88 +/- 63.23 uM	Children (1-13 years old)	Both	Obese	23108202	details
Blood	Detected and Quantified	142.2 (60.4) uM	Adult (>18 years old)	Female	Down syndrome pregnancy	23313728	details
Blood	Detected and Quantified	134.0 (52.4) uM	Adult (>18 years old)	Female	Pregnancy	23313728	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0 uM	Infant (0-1 year old)	Male	Dicarboxylic Aminoaciduria	894411	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.68 +/- 0.35 uM	Adult (>18 years old)	Both	Alzheimer's disease	17031479	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	27015276	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	23940645	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal Cancer	27275383	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Both	Autism	24130822	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Immunoglobulin A nephropathy (IgAN) progressor	26476344	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	ankylosing spondylitis	Tie-juan Sha...	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	rheumatoid arthritis	Tie-juan Sha...	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Both	celiac disease	21970810	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 (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	Irritable bowel syndrome	21761941	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Ulcerative colitis	21761941	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	28587349	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25105552	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	19006102	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Not Specified	celiac disease	27452636	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal Cancer	27107423	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Not Specified	Treated celiac disease	21970810	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	23940645	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Attachment loss	31026179	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Missing teeth	31026179	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Periodontal Probing Depth	31026179	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Tooth Decay	31026179	details
Saliva	Detected and Quantified	77.33 +/- 4.56 uM	Adult (>18 years old)	Male	Alzheimer's disease	23857558	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	48.08 +/- 21.24 uM	Adult (>18 years old)	Male	Frontotemporal lobe dementia	23857558	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Oral squamous cell carcinoma (OSCC)	21190195	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Oral leukoplakia (OLK)	21190195	details
Saliva	Detected and Quantified	72.05 +/- 35.86 uM	Adult (>18 years old)	Both	Lewy body disease	23857558	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Bladder cancer	28157703	details
Urine	Detected and Quantified	32.039 +/- 36.94 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details
Urine	Detected and Quantified	0.0068 - 0.0549 umol/mmol creatinine	Adult (>18 years old)	Both	ADPKD	21389975	details
Urine	Detected and Quantified	0 umol/mmol creatinine	Adolescent (13-18 years old)	Both	Hyperglycinuria	19033659	details
Urine	Detected and Quantified	0-1 umol/mmol creatinine	Adult (>18 years old)	Both	Hyperglycinuria	19033659	details
Urine	Detected and Quantified	0-1037 umol/mmol creatinine	Children (1-13 years old)	Both	Iminoglycinuria	19033659	details
Urine	Detected and Quantified	163-259 umol/mmol creatinine	Adult (>18 years old)	Female	Iminoglycinuria	19033659	details
Urine	Detected and Quantified	29.6 (19.7-39.47) umol/mmol creatinine	Children (1-13 years old)	Both	Iminoglycinuria	MetaGene: Metabol...	details
Urine	Detected and Quantified	3.6454 +/- 3.3038 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details
Urine	Detected and Quantified	2.6335 +/- 1.9039 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Gastroesophageal reflux disease	Analysis of 30 no...	details

Associated Disorders and Diseases

Disease References

Hemodialysis
Raj DS, Ouwendyk M, Francoeur R, Pierratos A: Plasma amino acid profile on nocturnal hemodialysis. Blood Purif. 2000;18(2):97-102. [PubMed:10838467 ]
Glutathione synthetase deficiency
G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
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 ]
Obesity
Reinehr T, Wolters B, Knop C, Lass N, Hellmuth C, Harder U, Peissner W, Wahl S, Grallert H, Adamski J, Illig T, Prehn C, Yu Z, Wang-Sattler R, Koletzko B: Changes in the serum metabolite profile in obese children with weight loss. Eur J Nutr. 2015 Mar;54(2):173-81. doi: 10.1007/s00394-014-0698-8. Epub 2014 Apr 17. [PubMed:24740590 ] Wahl S, Yu Z, Kleber M, Singmann P, Holzapfel C, He Y, Mittelstrass K, Polonikov A, Prehn C, Romisch-Margl W, Adamski J, Suhre K, Grallert H, Illig T, Wang-Sattler R, Reinehr T: Childhood obesity is associated with changes in the serum metabolite profile. Obes Facts. 2012;5(5):660-70. doi: 10.1159/000343204. Epub 2012 Oct 4. [PubMed:23108202 ] 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.
Hyperprolinemia, type I
Schafer IA, Scriver CR, Efron ML: Familial hyperprolinemia, cerebral dysfunction and renal anomalies occurring in a family with hereditary nephropathy and deafness. N Engl J Med. 1962 Jul 12;267:51-60. doi: 10.1056/NEJM196207122670201. [PubMed:14497974 ]
Dihydrolipoamide Dehydrogenase Deficiency
Kuhara T, Shinka T, Inoue Y, Matsumoto M, Yoshino M, Sakaguchi Y, Matsumoto I: Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clin Chim Acta. 1983 Sep 30;133(2):133-40. [PubMed:6688766 ]
Hyperprolinemia, type II
Mitsubuchi H, Nakamura K, Matsumoto S, Endo F: Biochemical and clinical features of hereditary hyperprolinemia. Pediatr Int. 2014 Aug;56(4):492-6. doi: 10.1111/ped.12420. [PubMed:24931297 ]
Pyruvate carboxylase deficiency
Habarou F, Brassier A, Rio M, Chretien D, Monnot S, Barbier V, Barouki R, Bonnefont JP, Boddaert N, Chadefaux-Vekemans B, Le Moyec L, Bastin J, Ottolenghi C, de Lonlay P: Pyruvate carboxylase deficiency: An underestimated cause of lactic acidosis. Mol Genet Metab Rep. 2014 Nov 28;2:25-31. doi: 10.1016/j.ymgmr.2014.11.001. eCollection 2015 Mar. [PubMed:28649521 ]
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 ]
Dicarboxylic aminoaciduria
Melancon SB, Dallaire L, Lemieux B, Robitaille P, Potier M: Dicarboxylic aminoaciduria: an inborn error of amino acid conservation. J Pediatr. 1977 Sep;91(3):422-7. [PubMed:894411 ]
Mitochondrial pyruvate carrier deficiency
Brivet M, Garcia-Cazorla A, Lyonnet S, Dumez Y, Nassogne MC, Slama A, Boutron A, Touati G, Legrand A, Saudubray JM: Impaired mitochondrial pyruvate importation in a patient and a fetus at risk. Mol Genet Metab. 2003 Mar;78(3):186-92. [PubMed:12649063 ]
Lipoyltransferase 1 Deficiency
Soreze Y, Boutron A, Habarou F, Barnerias C, Nonnenmacher L, Delpech H, Mamoune A, Chretien D, Hubert L, Bole-Feysot C, Nitschke P, Correia I, Sardet C, Boddaert N, Hamel Y, Delahodde A, Ottolenghi C, de Lonlay P: Mutations in human lipoyltransferase gene LIPT1 cause a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase. Orphanet J Rare Dis. 2013 Dec 17;8:192. doi: 10.1186/1750-1172-8-192. [PubMed:24341803 ]
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 ]
Colorectal cancer
Monleon D, Morales JM, Barrasa A, Lopez JA, Vazquez C, Celda B: Metabolite profiling of fecal water extracts from human colorectal cancer. NMR Biomed. 2009 Apr;22(3):342-8. doi: 10.1002/nbm.1345. [PubMed:19006102 ] Weir TL, Manter DK, Sheflin AM, Barnett BA, Heuberger AL, Ryan EP: Stool microbiome and metabolome differences between colorectal cancer patients and healthy adults. PLoS One. 2013 Aug 6;8(8):e70803. doi: 10.1371/journal.pone.0070803. Print 2013. [PubMed:23940645 ] 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 ] Lin Y, Ma C, Liu C, Wang Z, Yang J, Liu X, Shen Z, Wu R: NMR-based fecal metabolomics fingerprinting as predictors of earlier diagnosis in patients with colorectal cancer. Oncotarget. 2016 May 17;7(20):29454-64. doi: 10.18632/oncotarget.8762. [PubMed:27107423 ] 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 ] 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 ] Wang X, Wang J, Rao B, Deng L: Gut flora profiling and fecal metabolite composition of colorectal cancer patients and healthy individuals. Exp Ther Med. 2017 Jun;13(6):2848-2854. doi: 10.3892/etm.2017.4367. Epub 2017 Apr 20. [PubMed:28587349 ]
Irritable bowel syndrome
Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ]
Ulcerative colitis
Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ] 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 ]
Celiac disease
Di Cagno R, De Angelis M, De Pasquale I, Ndagijimana M, Vernocchi P, Ricciuti P, Gagliardi F, Laghi L, Crecchio C, Guerzoni ME, Gobbetti M, Francavilla R: Duodenal and faecal microbiota of celiac children: molecular, phenotype and metabolome characterization. BMC Microbiol. 2011 Oct 4;11:219. doi: 10.1186/1471-2180-11-219. [PubMed:21970810 ] De Angelis M, Vannini L, Di Cagno R, Cavallo N, Minervini F, Francavilla R, Ercolini D, Gobbetti M: Salivary and fecal microbiota and metabolome of celiac children under gluten-free diet. Int J Food Microbiol. 2016 Dec 19;239:125-132. doi: 10.1016/j.ijfoodmicro.2016.07.025. Epub 2016 Jul 19. [PubMed:27452636 ]
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 ]
Rheumatoid arthritis
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]
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 ]
Attachment loss
Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Missing teeth
Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Periodontal Probing Depth
Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Tooth Decay
Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Iminoglycinuria
Lasley L, Scriver CR: Ontogeny of amino acid reabsorption in human kidney. Evidence from the homozygous infant with familial renal iminoglycinuria for multiple proline and glycine systems. Pediatr Res. 1979 Jan;13(1):65-70. [PubMed:432003 ] Broer S, Bailey CG, Kowalczuk S, Ng C, Vanslambrouck JM, Rodgers H, Auray-Blais C, Cavanaugh JA, Broer A, Rasko JE: Iminoglycinuria and hyperglycinuria are discrete human phenotypes resulting from complex mutations in proline and glycine transporters. J Clin Invest. 2008 Dec;118(12):3881-92. doi: 10.1172/JCI36625. Epub 2008 Nov 6. [PubMed:19033659 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Autosomal dominant polycystic kidney disease
Gronwald W, Klein MS, Zeltner R, Schulze BD, Reinhold SW, Deutschmann M, Immervoll AK, Boger CA, Banas B, Eckardt KU, Oefner PJ: Detection of autosomal dominant polycystic kidney disease by NMR spectroscopic fingerprinting of urine. Kidney Int. 2011 Jun;79(11):1244-53. doi: 10.1038/ki.2011.30. Epub 2011 Mar 9. [PubMed:21389975 ]
Eosinophilic esophagitis
Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.

Associated OMIM IDs

266130 (Glutathione synthetase deficiency)
601665 (Obesity)
239500 (Hyperprolinemia, type I)
246900 (Dihydrolipoamide Dehydrogenase Deficiency)
239510 (Hyperprolinemia, type II)
266150 (Pyruvate carboxylase deficiency)
219150 (Cutis laxa, autosomal recessive, type IIIA)
222730 (Dicarboxylic aminoaciduria)
614741 (Mitochondrial pyruvate carrier deficiency)
616299 (Lipoyltransferase 1 Deficiency)
104300 (Alzheimer's disease)
114500 (Colorectal cancer)
212750 (Celiac disease)
209850 (Autism)
266600 (Crohn's disease)
180300 (Rheumatoid arthritis)
260350 (Pancreatic cancer)
170650 (Periodontal disease)
600274 (Frontotemporal dementia)
242600 (Iminoglycinuria)
601313 (Autosomal dominant polycystic kidney disease)
610247 (Eosinophilic esophagitis)

External Links

DrugBank ID

DB00172

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

145742

PDB ID

Not Available

ChEBI ID

17203

Food Biomarker Ontology

Not Available

VMH ID

PRO_L

MarkerDB ID

MDB00000079

Good Scents ID

Not Available

References

Synthesis Reference

Itoh, Tamio. Synthesis of L-proline from L-glutamine. Bulletin of the Chemical Society of Japan (1963), 36(1), 25-9

Material Safety Data Sheet (MSDS)

Not Available

General References

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 ]
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 ]
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 ]
Deng C, Shang C, Hu Y, Zhang X: Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):115-20. [PubMed:12101068 ]
Azizi M, Junot C, Ezan E, Menard J: Angiotensin I-converting enzyme and metabolism of the haematological peptide N-acetyl-seryl-aspartyl-lysyl-proline. Clin Exp Pharmacol Physiol. 2001 Dec;28(12):1066-9. [PubMed:11903317 ]
Roesel RA, Blankenship PR, Mobley EB, Coryell ME: Increased excretion of histidyl-L-proline diketopiperazine by infants receiving Pregestimil and Nutramigen formulas. Clin Chem. 1986 May;32(5):865-7. [PubMed:3698278 ]
Chambers ST, Kunin CM: Osmoprotective activity for Escherichia coli in mammalian renal inner medulla and urine. Correlation of glycine and proline betaines and sorbitol with response to osmotic loads. J Clin Invest. 1987 Nov;80(5):1255-60. [PubMed:3316273 ]
Azizi M, Ezan E, Reny JL, Wdzieczak-Bakala J, Gerineau V, Menard J: Renal and metabolic clearance of N-acetyl-seryl-aspartyl-lysyl-proline (AcSDKP) during angiotensin-converting enzyme inhibition in humans. Hypertension. 1999 Mar;33(3):879-86. [PubMed:10082503 ]
Hart W, van den Hamer CJ, van der Sluys Veer J: The use of hydroxy-DL-proline-2-(14)C in the investigation of hydroxyproline metabolism in normal subjects and in patients with renal insufficiency. Clin Nephrol. 1976 Sep;6(3):379-87. [PubMed:991465 ]
Kanwar YS, Krakower CA, Manaligod JR, Justice P, Wong PW: Biochemical, morphological and hybrid studies in hyperprolinemic mice. Biomedicine. 1975 May;22(3):209-16. [PubMed:240452 ]
Simila S: Hydroxyproline metabolism in type II hyperprolinaemia. Ann Clin Biochem. 1979 Jul;16(4):177-81. [PubMed:533224 ]
Lu SH, Ohshima H, Fu HM, Tian Y, Li FM, Blettner M, Wahrendorf J, Bartsch H: Urinary excretion of N-nitrosamino acids and nitrate by inhabitants of high- and low-risk areas for esophageal cancer in Northern China: endogenous formation of nitrosoproline and its inhibition by vitamin C. Cancer Res. 1986 Mar;46(3):1485-91. [PubMed:3943105 ]
Hausmann D: [Post-traumatic imbalances of plasma amino acids--interference factors or defense mechanisms? A study of protein metabolism in severe craniocerebral trauma]. Anasth Intensivther Notfallmed. 1988 Feb;23(1):14-21. [PubMed:3364629 ]
Scriver CR, McInnes RR, Mohyuddin F: Role of epithelial architecture and intracellular metabolism in proline uptake and transtubular reclamation in PRO/re mouse kidney. Proc Natl Acad Sci U S A. 1975 Apr;72(4):1431-5. [PubMed:1055415 ]
Boden G, Rezvani I, Owen OE: Effects of glucagon on plasma amino acids. J Clin Invest. 1984 Mar;73(3):785-93. [PubMed:6142902 ]
Feldman JM, Plonk JW, Admiraal J, Sidbury JB: Plasma amino acids in patients with the carcinoid syndrome. Cancer. 1976 Nov;38(5):2127-31. [PubMed:136295 ]
Humbertclaude V, Rivier F, Roubertie A, Echenne B, Bellet H, Vallat C, Morin D: Is hyperprolinemia type I actually a benign trait? Report of a case with severe neurologic involvement and vigabatrin intolerance. J Child Neurol. 2001 Aug;16(8):622-3. [PubMed:11510941 ]
Nagata Y, Higashi M, Ishii Y, Sano H, Tanigawa M, Nagata K, Noguchi K, Urade M: The presence of high concentrations of free D-amino acids in human saliva. Life Sci. 2006 Mar 6;78(15):1677-81. Epub 2006 Feb 9. [PubMed:16480744 ]
Nagata Y, Masui R, Akino T: The presence of free D-serine, D-alanine and D-proline in human plasma. Experientia. 1992 Oct 15;48(10):986-8. [PubMed:1426150 ]
Nadler JV, Wang A, Hakim A: Toxicity of L-proline toward rat hippocampal neurons. Brain Res. 1988 Jul 19;456(1):168-72. [PubMed:3409032 ]
Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

Only showing the first 10 proteins. There are 30 proteins in total.

Show all enzymes and transporters

Enzymes

Enzyme Details

1. Prolyl 4-hydroxylase subunit alpha-2

General function:: Involved in oxidoreductase activity
Specific function:: Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
Gene Name:: P4HA2
Uniprot ID:: O15460
Molecular weight:: 60632.19

Reactions

Proline + Oxoglutaric acid + Oxygen → 4-Hydroxyproline + Succinic acid + Carbon dioxide	details

References

Enzyme Details

2. Pyrroline-5-carboxylate reductase 1, mitochondrial

General function:: Involved in oxidoreductase activity
Specific function:: Housekeeping enzyme that catalyzes the last step in proline biosynthesis. Can utilize both NAD and NADP, but has higher affinity for NAD. Involved in the cellular response to oxidative stress.
Gene Name:: PYCR1
Uniprot ID:: P32322
Molecular weight:: 33360.27

Reactions

Proline + NAD(P)(+) → 1-Pyrroline-5-carboxylic acid + NAD(P)H	details
Proline + NAD → 1-Pyrroline-5-carboxylic acid + NADH + Hydrogen Ion	details
Proline + NADP → 1-Pyrroline-5-carboxylic acid + NADPH + Hydrogen Ion	details

References

Enzyme Details

3. Prolyl 4-hydroxylase subunit alpha-1

General function:: Involved in oxidoreductase activity
Specific function:: Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
Gene Name:: P4HA1
Uniprot ID:: P13674
Molecular weight:: 60966.645

Reactions

Proline + Oxoglutaric acid + Oxygen → 4-Hydroxyproline + Succinic acid + Carbon dioxide	details

References

Enzyme Details

4. Pyrroline-5-carboxylate reductase 2

General function:: Involved in oxidoreductase activity
Specific function:: Housekeeping enzyme that catalyzes the last step in proline biosynthesis. In some cell types, such as erythrocytes, its primary function may be the generation of NADP(+). Can utilize both NAD and NADP. Has higher affinity for NADP, but higher catalytic efficiency with NADH.
Gene Name:: PYCR2
Uniprot ID:: Q96C36
Molecular weight:: 25867.975

Reactions

Proline + NAD(P)(+) → 1-Pyrroline-5-carboxylic acid + NAD(P)H	details
Proline + NAD → 1-Pyrroline-5-carboxylic acid + NADH + Hydrogen Ion	details
Proline + NADP → 1-Pyrroline-5-carboxylic acid + NADPH + Hydrogen Ion	details

References

Enzyme Details

5. Bifunctional glutamate/proline--tRNA ligase

General function:: Involved in nucleotide binding
Specific function:: Catalyzes the attachment of the cognate amino acid to the corresponding tRNA in a two-step reaction: the amino acid is first activated by ATP to form a covalent intermediate with AMP and is then transferred to the acceptor end of the cognate tRNA. Component of the GAIT (gamma interferon-activated inhibitor of translation) complex which mediates interferon-gamma-induced transcript-selective translation inhibition in inflammation processes. Upon interferon-gamma activation and subsequent phosphorylation dissociates from the multisynthetase complex and assembles into the GAIT complex which binds to stem loop-containing GAIT elements in the 3'-UTR of diverse inflammatory mRNAs (such as ceruplasmin) and suppresses their translation.
Gene Name:: EPRS
Uniprot ID:: P07814
Molecular weight:: 170589.705

Reactions

Adenosine triphosphate + Proline + tRNA(Pro) → Adenosine monophosphate + Pyrophosphate + L-prolyl-tRNA(Pro)	details
Adenosine triphosphate + Proline + tRNA(Pro) → Adenosine monophosphate + Pyrophosphate + L-Prolyl-tRNA(Pro)	details

References

Enzyme Details

6. Cytosol aminopeptidase

General function:: Involved in aminopeptidase activity
Specific function:: Presumably involved in the processing and regular turnover of intracellular proteins. Catalyzes the removal of unsubstituted N-terminal amino acids from various peptides.
Gene Name:: LAP3
Uniprot ID:: P28838
Molecular weight:: Not Available

Reactions

Peptide + Water → Proline + Peptide	details

Enzyme Details

7. Proline dehydrogenase 1, mitochondrial

General function:: Involved in proline dehydrogenase activity
Specific function:: Converts proline to delta-1-pyrroline-5-carboxylate.
Gene Name:: PRODH
Uniprot ID:: O43272
Molecular weight:: 56196.675

Reactions

Proline + acceptor → 1-Pyrroline-5-carboxylic acid + reduced acceptor	details
Proline + Acceptor → 1-Pyrroline-5-carboxylic acid + Reduced acceptor	details

References

Enzyme Details

8. Pyrroline-5-carboxylate reductase 3

General function:: Involved in oxidoreductase activity
Specific function:: Not Available
Gene Name:: PYCRL
Uniprot ID:: Q53H96
Molecular weight:: 29892.415

Reactions

Proline + NAD(P)(+) → 1-Pyrroline-5-carboxylic acid + NAD(P)H	details
Proline + NAD → 1-Pyrroline-5-carboxylic acid + NADH + Hydrogen Ion	details
Proline + NADP → 1-Pyrroline-5-carboxylic acid + NADPH + Hydrogen Ion	details

References

Enzyme Details

9. Proline synthase co-transcribed bacterial homolog protein

General function:: Not Available
Specific function:: Not Available
Gene Name:: PROSC
Uniprot ID:: O94903
Molecular weight:: 30343.7

References

Enzyme Details

10. Pyrroline-5-carboxylate reductase

General function:: Involved in oxidoreductase activity
Specific function:: L-proline + NAD(P)(+) = 1-pyrroline-5- carboxylate + NAD(P)H
Gene Name:: P5CR2
Uniprot ID:: Q4W8W1
Molecular weight:: 33726.4

References

Transporters

Enzyme Details

1. Monocarboxylate transporter 10

General function:: Involved in transmembrane transport
Specific function:: Sodium-independent transporter that mediates the update of aromatic acid. Can function as a net efflux pathway for aromatic amino acids in the basosolateral epithelial cells
Gene Name:: SLC16A10
Uniprot ID:: Q8TF71
Molecular weight:: 55492.1

References

Only showing the first 10 proteins. There are 30 proteins in total.

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Showing metabocard for Proline (HMDB0000162)

MOL for HMDB0000162 (Proline)

3D MOL for HMDB0000162 (Proline)

3D SDF for HMDB0000162 (Proline)

3D-SDF for HMDB0000162 (Proline)

PDB for HMDB0000162 (Proline)

3D PDB for HMDB0000162 (Proline)

SMILES for HMDB0000162 (Proline)

INCHI for HMDB0000162 (Proline)

Structure for HMDB0000162 (Proline)

3D Structure for HMDB0000162 (Proline)

Experimental Collision Cross Sections

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

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MS/MS Spectra

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IR Spectra

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