Jump To Section:

Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2023-05-30 20:55:50 UTC

HMDB ID

HMDB0000070

Secondary Accession Numbers

HMDB00070

Metabolite Identification

Common Name

Pipecolic acid

Description

Pipecolic acid is a metabolite of lysine found in human physiological fluids such as urine, plasma and CSF. However, it is uncertain if pipecolic acid originates directly from food intake or from mammalian or intestinal bacterial enzyme metabolism. Recent studies suggest that plasma pipecolic acid, particularly the D-isomer, originates mainly from the catabolism of dietary lysine by intestinal bacteria rather than by direct food intake. In classic Zellweger syndrome (a cerebro-hepato-renal genetic disorder, OMIM 214100 ) pipecolic acid accumulate in the plasma of the patients. It is known that plasma pipecolic acid levels are also elevated in patients with chronic liver diseases. Pipecolic acid is moderately elevated in patients with pyridoxine-dependent seizures and might therefore be a possible biochemical marker for selecting candidates for pyridoxine therapy (Plecko et al 2000). Pipecolic acid was also elevated in CSF in these vitamin B6-responsive patients (PMID 12705501 ). Pipecolic acid is found to be associated with adrenoleukodystrophy, infantile Refsum disease, and peroxisomal biogenesis defect, which are also inborn errors of metabolism. Pipecolic acid is a biomarker for the consumption of dried and cooked beans.

Structure

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

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
2-Piperidinecarboxylic acid	ChEBI
Homoproline	ChEBI
Pipecolinic acid	ChEBI
2-Piperidinecarboxylate	Generator
Pipecolinate	Generator
Pipecolate	Generator
()-Piperidine-2-carboxylic acid	HMDB
(+/-)-2-piperidinecarboxylate	HMDB
(+/-)-2-piperidinecarboxylic acid	HMDB
(+/-)-pipecolate	HMDB
(+/-)-pipecolic acid	HMDB
(+/-)-pipecolinate	HMDB
(+/-)-pipecolinic acid	HMDB
(.+/-.)-2-piperidinecarboxylic acid	HMDB
(RS)-2-Piperidinecarboxylate	HMDB
(RS)-2-Piperidinecarboxylic acid	HMDB
.alpha.-pipecolinic acid	HMDB
2-Carboxypiperidine	HMDB
2-Pipecolinic acid	HMDB
2-Piperidinylcarboxylic acid	HMDB
a-Pipecolinate	HMDB
a-Pipecolinic acid	HMDB
Acide pipecolique	HMDB
Acide piperidine-carboxylique-2	HMDB
alpha-Pipecolinate	HMDB
alpha-Pipecolinic acid	HMDB
Dihydrobaikiane	HMDB
DL-2-Piperidinecarboxylate	HMDB
DL-2-Piperidinecarboxylic acid	HMDB
DL-Homoproline	HMDB
DL-Pipecolate	HMDB, Generator
DL-Pipecolic acid	HMDB
DL-Pipecolinate	HMDB
DL-Pipecolinic acid	HMDB
hexahydro-2-Picolinate	HMDB
hexahydro-2-Picolinic acid	HMDB
Hexahydropicolinate	HMDB
Hexahydropicolinic acid	HMDB
Pipecolic acid free base	HMDB
Piperidine-2-carboxylic acid	HMDB
Piperolinate	HMDB
Piperolinic acid	HMDB
L-Pipecolic acid	MeSH, HMDB
Pipecolic acid, (R)-isomer	MeSH, HMDB
Pipecolic acid, ion (1-)	MeSH, HMDB
Pipecolic acid, monopotassium salt	MeSH, HMDB
Pipecolic acid hydrochloride, (+-)-isomer	MeSH, HMDB
Pipecolic acid, (S)-isomer	MeSH, HMDB
Pipecolic acid, 14C-labeled CPD, (+,-)-isomer	MeSH, HMDB
Homopipecolic acid	MeSH, HMDB
Pipecolic acid, (+,-)-isomer	MeSH, HMDB
Pipecolic acid, ion(1-), (+,-)-isomer	MeSH, HMDB
Pipecolic acid, ion(1-), (S)-isomer	MeSH, HMDB
Piperidine-2-carboxylate	Generator, HMDB
Pipecolic acid	MeSH

Chemical Formula

C₆H₁₁NO₂

Average Molecular Weight

129.157

Monoisotopic Molecular Weight

129.078978601

IUPAC Name

piperidine-2-carboxylic acid

Traditional Name

(+,-)-pipecolic acid

CAS Registry Number

535-75-1

SMILES

OC(=O)C1CCCCN1

InChI Identifier

InChI=1S/C6H11NO2/c8-6(9)5-3-1-2-4-7-5/h5,7H,1-4H2,(H,8,9)

InChI Key

HXEACLLIILLPRG-UHFFFAOYSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as alpha amino acids. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon).

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

Alpha amino acids

Alternative Parents

Substituents

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

Molecular Framework

Aliphatic heteromonocyclic compounds

External Descriptors

piperidinemonocarboxylic acid (CHEBI:17964 )

Ontology

Not Available

Exogenous (HMDB: HMDB0062767)

Process

Not Available

Role

Not Available

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	264 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	314 mg/mL	Not Available
LogP	-2.31	TSAI,RS ET AL. (1991)

Experimental Chromatographic Properties

Not Available

Predicted Molecular Properties

Property	Value	Source
Water Solubility	158 g/L	ALOGPS
logP	-2.2	ALOGPS
logP	-2.1	ChemAxon
logS	0.09	ALOGPS
pKa (Strongest Acidic)	2.06	ChemAxon
pKa (Strongest Basic)	10.39	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	32.67 m³·mol⁻¹	ChemAxon
Polarizability	13.47 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]+	126.796	31661259
DarkChem	[M-H]-	119.67	31661259
DarkChem	[M+H]+	126.796	31661259
DarkChem	[M-H]-	119.67	31661259
AllCCS	[M+H]+	127.77	32859911
AllCCS	[M-H]-	125.31	32859911
DeepCCS	[M+H]+	125.823	30932474
DeepCCS	[M-H]-	122.372	30932474
DeepCCS	[M-2H]-	159.391	30932474
DeepCCS	[M+Na]+	134.595	30932474
AllCCS	[M+H]+	127.8	32859911
AllCCS	[M+H-H2O]+	123.0	32859911
AllCCS	[M+NH4]+	132.2	32859911
AllCCS	[M+Na]+	133.5	32859911
AllCCS	[M-H]-	125.3	32859911
AllCCS	[M+Na-2H]-	127.4	32859911
AllCCS	[M+HCOO]-	129.7	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
Pipecolic acid	OC(=O)C1CCCCN1	1881.1	Standard polar	33892256
Pipecolic acid	OC(=O)C1CCCCN1	1156.3	Standard non polar	33892256
Pipecolic acid	OC(=O)C1CCCCN1	1256.0	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
Pipecolic acid,1TMS,isomer #1	C[Si](C)(C)OC(=O)C1CCCCN1	1263.2	Semi standard non polar	33892256
Pipecolic acid,1TMS,isomer #2	C[Si](C)(C)N1CCCCC1C(=O)O	1344.1	Semi standard non polar	33892256
Pipecolic acid,2TMS,isomer #1	C[Si](C)(C)OC(=O)C1CCCCN1[Si](C)(C)C	1356.9	Semi standard non polar	33892256
Pipecolic acid,2TMS,isomer #1	C[Si](C)(C)OC(=O)C1CCCCN1[Si](C)(C)C	1394.2	Standard non polar	33892256
Pipecolic acid,2TMS,isomer #1	C[Si](C)(C)OC(=O)C1CCCCN1[Si](C)(C)C	1694.1	Standard polar	33892256
Pipecolic acid,1TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)C1CCCCN1	1497.9	Semi standard non polar	33892256
Pipecolic acid,1TBDMS,isomer #2	CC(C)(C)[Si](C)(C)N1CCCCC1C(=O)O	1572.1	Semi standard non polar	33892256
Pipecolic acid,2TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)C1CCCCN1[Si](C)(C)C(C)(C)C	1842.5	Semi standard non polar	33892256
Pipecolic acid,2TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)C1CCCCN1[Si](C)(C)C(C)(C)C	1813.2	Standard non polar	33892256
Pipecolic acid,2TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)C1CCCCN1[Si](C)(C)C(C)(C)C	1963.5	Standard polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental GC-MS	GC-MS Spectrum - Pipecolic acid GC-EI-TOF (Non-derivatized)	splash10-0a4i-0910000000-d9cfd443adb5187a3f21	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Pipecolic acid GC-EI-TOF (Non-derivatized)	splash10-0a4i-0910000000-d9cfd443adb5187a3f21	2018-05-18	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Pipecolic acid GC-EI-TOF (Non-derivatized)	splash10-0a4i-0910000000-d9cfd443adb5187a3f21	2018-05-18	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Pipecolic acid GC-EI-TOF (Non-derivatized)	splash10-0a4i-0910000000-d9cfd443adb5187a3f21	2018-05-18	HMDB team, MONA, MassBank	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Pipecolic acid GC-MS (Non-derivatized) - 70eV, Positive	splash10-001i-9200000000-5c4f6059bcc868ca23fd	2016-09-22	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Pipecolic acid GC-MS (1 TMS) - 70eV, Positive	splash10-05fu-9700000000-8003521353d4d37af49f	2017-10-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Pipecolic acid GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Pipecolic acid GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Pipecolic acid GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Pipecolic acid 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 - Pipecolic acid Quattro_QQQ 10V, Positive-QTOF (Annotated)	splash10-0060-9900000000-c5b5bf28424d5d5d1bda	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Pipecolic acid Quattro_QQQ 25V, Positive-QTOF (Annotated)	splash10-001i-9000000000-92660ad26bff36fba7ef	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Pipecolic acid Quattro_QQQ 40V, Positive-QTOF (Annotated)	splash10-0a59-9000000000-b779372fa816467754c1	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Pipecolic acid LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positive-QTOF	splash10-001i-9300000000-26dee47121401990f1c1	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Pipecolic acid LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) 30V, Positive-QTOF	splash10-001i-9000000000-929057dfd61546bee24d	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Pipecolic acid LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative-QTOF	splash10-004i-0900000000-6595801dc6a322f712f0	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Pipecolic acid LC-ESI-QTOF , negative-QTOF	splash10-004i-0900000000-6595801dc6a322f712f0	2017-09-14	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Pipecolic acid , negative-QTOF	splash10-004i-0900000000-99a7a942cee58c76115a	2017-09-14	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Pipecolic acid LC-ESI-QTOF , positive-QTOF	splash10-001i-9300000000-26dee47121401990f1c1	2017-09-14	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Pipecolic acid LC-ESI-QTOF , positive-QTOF	splash10-001i-9000000000-929057dfd61546bee24d	2017-09-14	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Pipecolic acid , positive-QTOF	splash10-001i-9600000000-9631d0fbc984509f8d1e	2017-09-14	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Pipecolic acid 30V, Positive-QTOF	splash10-001i-9000000000-b244255a58ffb3b92be5	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Pipecolic acid 35V, Positive-QTOF	splash10-001i-9000000000-c3041e7b5b1f5118c1ec	2021-09-20	HMDB team, MONA	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Pipecolic acid 10V, Positive-QTOF	splash10-001i-3900000000-b2ceb4c88f50a6524604	2015-05-27	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Pipecolic acid 20V, Positive-QTOF	splash10-001i-9400000000-d3fab76e3fbfbe92c14d	2015-05-27	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Pipecolic acid 40V, Positive-QTOF	splash10-0a5c-9000000000-20464a5c62992da01ab7	2015-05-27	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Pipecolic acid 10V, Negative-QTOF	splash10-004i-2900000000-6fac2bf48ffe4d910118	2015-05-27	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Pipecolic acid 20V, Negative-QTOF	splash10-003r-8900000000-81b6d69c7a568e36b49f	2015-05-27	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Pipecolic acid 40V, Negative-QTOF	splash10-001i-9000000000-6332d1986eb0da0057fd	2015-05-27	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Pipecolic acid 10V, Negative-QTOF	splash10-004i-0900000000-b487cd2a72f17977a8cd	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Pipecolic acid 20V, Negative-QTOF	splash10-01t9-1900000000-83b59287dc6d3e726326	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Pipecolic acid 40V, Negative-QTOF	splash10-0036-9200000000-3ca813e7348e686ed922	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Pipecolic acid 10V, Positive-QTOF	splash10-001i-9300000000-cb8df30f7267d610fd8d	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Pipecolic acid 20V, Positive-QTOF	splash10-001i-9000000000-72c8691eb15dce6e0abb	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Pipecolic acid 40V, Positive-QTOF	splash10-053r-9000000000-c7623c5058ad6c879ec3	2021-09-22	Wishart Lab	View Spectrum

NMR Spectra

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

IR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted IR Spectrum	IR Ion Spectrum (Quadrupole Ion Trap, ESI+, Adduct: [M+H]+)	2022-02-11	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+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

Biospecimen Locations

Amniotic Fluid
Blood
Cerebrospinal Fluid (CSF)
Feces
Saliva
Urine

Tissue Locations

Intestine
Liver
Placenta
Prostate

Pathways

Not Available

Name	SMPDB/PathBank	KEGG

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Amniotic Fluid	Detected and Quantified	4.65 (2.24 - 8.40) uM	Not Specified	Both	Normal	3315316	details
Blood	Detected and Quantified	2.46 +/- 1.26 uM	Adult (>18 years old)	Both	Normal	12705501	details
Blood	Detected and Quantified	0-5 uM	Newborn (0-30 days old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	0-5 uM	Infant (1 - 3 months old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	5.73 (3.75 - 10.8) uM	Newborn (0-30 days old)	Both	Normal	3315316	details
Blood	Detected and Quantified	0-10 uM	Children (3 months - 6 years old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	0-10 uM	Children (6 - 18 years old)	Both	Normal	BC Children's Hos...	details
Blood	Detected and Quantified	1.46 (0.70-2.46) uM	Newborn (0-30 days old)	Both	Normal	3315316	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Blood	Detected and Quantified	<4 uM	Children (1-13 years old)	Not Specified	Normal	2894756	details
Blood	Detected and Quantified	2.0 +/- 0.8 uM	Infant (0-1 year old)	Not Specified	Normal	2921319	details
Blood	Detected and Quantified	0.4-4.9 uM	Infant (0-1 year old)	Not Specified	Normal	17068770	details
Blood	Detected and Quantified	<2.323 uM	Children (1 - 13 years old)	Male	Normal	2427795	details
Blood	Detected and Quantified	<3 uM	Children (1 - 13 years old)	Male	Normal	2427795	details
Blood	Detected and Quantified	0.540-2.460 uM	Newborn (0-30 days old)	Both	Normal	9818927	details
Blood	Detected and Quantified	0.774-54.198 uM	Children (1-13 years old)	Both	Normal	7931872	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	24789758	details
Blood	Detected and Quantified	10.0 (0.0-20.0) uM	Children (1-13 years old)	Both	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	1.20 +/- 0.11 uM	Adult (>18 years old)	Both	Normal	10509903	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Normal	27355821	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.75 (0.0-1.5) uM	Adult (>18 years old)	Both	Normal	2427795	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	<1.5 uM	Children (1 - 13 years old)	Male	Normal	2427795	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.056 (0.009 - 0.120) uM	Adult (>18 years old)	Both	Normal	12705501	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.08 (0.07-0.10) uM	Adult (>18 years old)	Not Specified	Normal	11865422	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.017 +/- 0.005 uM	Adult (>18 years old)	Not Specified	Normal	15206793	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	<1.6 uM	Children (1-13 years old)	Both	Normal	23890588	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0-0.15 uM	Children (0 - 10 years old)	Both	Normal	BC Children's Hos...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0-0.15 uM	Adolescent (>11 years old)	Both	Normal	BC Children's Hos...	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	20669995	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	24029555	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27543620	details
Feces	Detected and Quantified	61.166 +/- 74.328 nmol/g wet feces	Not Specified	Not Specified	Normal	20669995	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	29808030	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	20300169	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
Urine	Detected and Quantified	0.28 (0.0-0.56) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	2427795	details
Urine	Detected and Quantified	0.03 +/-0.07 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	10509903	details
Urine	Detected and Quantified	0.032 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	19309105	details
Urine	Detected and Quantified	<0.565 umol/mmol creatinine	Children (1 - 13 years old)	Male	Normal	2427795	details
Urine	Detected and Quantified	<6 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	2894756	details
Urine	Detected and Quantified	0.15 (0.14-0.24) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Analysis of 40 NI...	details
Urine	Detected and Quantified	< 0.566 umol/mmol creatinine	Not Available	Not Available	Normal	2427795	details
Urine	Detected and Quantified	0 - 25.33 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 13.91 umol/mmol creatinine	Infant (1 - 6 months old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 5.09 umol/mmol creatinine	Infant (6 months - <1 year old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 0.64 umol/mmol creatinine	Children (1 - 2 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 0.64 umol/mmol creatinine	Children (2 - 4 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 0.64 umol/mmol creatinine	Children (4 - 13 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 0.64 umol/mmol creatinine	Adolescent (13 - 21 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0 - 0.64 umol/mmol creatinine	Adult (>21 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	0.03 +/- 0.07 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	2026685	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	22024767	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	39.6 uM	Infant (0-1 year old)	Both	Infantile Refsum disease	2427795	details
Blood	Detected and Quantified	79.0 (5.0 - 378.0) uM	Adult (>18 years old)	Both	Peroxisomal biogenesis defect	12705501	details
Blood	Detected and Quantified	2.8-23 uM	Infant (0-1 year old)	Not Specified	Pyridoxine dependency with seizures (PDE)	17068770	details
Blood	Detected and Quantified	2.3 uM	Infant (0-1 year old)	Male	Pseudo Zellweger->D-bifunctional protein deficiency	2921319	details
Blood	Detected and Quantified	0-1.66 uM	Children (1-13 years old)	Both	Pseudoneonatal adrenoleukodystrophy	2894756	details
Blood	Detected and Quantified	4.1 uM	Newborn (0-30 days old)	Female	Rhizomelic chondrodysplasia punctata	9818927	details
Blood	Detected and Quantified	9.22 uM	Newborn (0-30 days old)	Not Specified	Zellweger syndrome	9818927	details
Blood	Detected and Quantified	21 uM	Infant (0-1 year old)	Male	Peroxisomal biogenesis disorder	9818927	details
Blood	Detected and Quantified	<3.871 uM	Children (1-13 years old)	Not Specified	Adrenoleukodystrophy, X-linked	2408988	details
Blood	Detected and Quantified	<3.871 uM	Infant (0-1 year old)	Not Specified	Refsum disease, classic, adult-onset	2408988	details
Blood	Detected and Quantified	0-1.660 uM	Newborn (0-30 days old)	Not Specified	Neonatal adrenoleukodystrophy	9818927	details
Blood	Detected and Quantified	0-38.713 uM	Children (1-13 years old)	Not Specified	Refsum disease, juvenile	2408988	details
Blood	Detected and Quantified	15-64 uM	Children (1 - 13 years old)	Male	Infantile Refsum disease	2427795	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Schizophrenia	24789758	details
Blood	Detected and Quantified	170.335-185.820 uM	Children (1-13 years old)	Not Specified	Refsum disease, infantile	2408988	details
Blood	Detected and Quantified	232.275-363.898 uM	Children (1-13 years old)	Male	Peroxisomal disorders, new type, liver	7931872	details
Blood	Detected and Quantified	26.325-216.790 uM	Infant (0-1 year old)	Both	Zellweger syndrome	2418187	details
Blood	Detected and Quantified	27.0988-162.593 uM	Newborn (0-30 days old)	Both	Neonatal adrenoleukodystrophy	2408988	details
Blood	Detected and Quantified	38.712-154.85 uM	Children (1 - 13 years old)	Male	Infantile Refsum's disease	2427795	details
Blood	Detected and Quantified	4.120-280 uM	Newborn (0-30 days old)	Female	Peroxisomal biogenesis disorder	9818927	details
Blood	Detected and Quantified	60.860-62.100 uM	Children (1-13 years old)	Female	Peroxisomal biogenesis disorder	9818927	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	1.8 uM	Infant (0-1 year old)	Both	Infantile Refsum disease	2427795	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	<0.5 - 3.5 uM	Children (1 - 13 years old)	Male	Infantile Refsum disease	2427795	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.053 (0.023-0.083) uM	Adult (>18 years old)	Both	Brain injury	15206793	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.19 (0.17-0.21) uM	Adult (>18 years old)	Not Specified	Malaria	11865422	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.38 (0.11-1.26) uM	Not Specified	Not Specified	Anemia	12964115	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.18 (0.14-0.22) uM	Not Specified	Not Specified	Malaria	12964115	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.17 (0.12-0.23) uM	Not Specified	Not Specified	Tuberculous meningitis	12964115	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.06 (0.02-0.26) uM	Adult (>18 years old)	Not Specified	Malaria	12964115	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	24-33 uM	Children (1-13 years old)	Both	Hyperlysinemia I, familial	23890588	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.05 (0.02-0.09) uM	Children (1-13 years old)	Not Specified	Convulsions	12964115	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	1-2 uM	Children (1-13 years old)	Male	Hyperpipecolatemia	5700850	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	1.715 (0.33 - 3.10) uM	Adult (>18 years old)	Both	Peroxisomal biogenesis defect	10509903	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	25037050	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal Cancer	27275383	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)	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
Urine	Detected and Quantified	2.0 umol/mmol creatinine	Infant (0-1 year old)	Both	Infantile Refsum disease	2427795	details
Urine	Detected and Quantified	3.3 (1.5-5.0) umol/mmol creatinine	Adult (>18 years old)	Both	Adrenoleukodystrophy	MetaGene: Metabol...	details
Urine	Detected and Quantified	49.4 (8.4-90.4) umol/mmol creatinine	Children (1-13 years old)	Both	Adrenoleukodystrophy (ALD)	MetaGene: Metabol...	details
Urine	Detected and Quantified	0.02-228.3 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Zellweger Syndrome	3769211	details
Urine	Detected and Quantified	0-5.314 umol/mmol creatinine	Children (1 - 13 years old)	Male	Infantile Refsum disease	2427795	details
Urine	Detected and Quantified	0.93-70 umol/mmol creatinine	Children (1-13 years old)	Both	Pseudoneonatal adrenoleukodystrophy	2894756	details
Urine	Detected and Quantified	4.522 umol/mmol creatinine	Children (1-13 years old)	Male	Hyperpipecolatemia	5700850	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Schizophrenia	22024767	details

Associated Disorders and Diseases

Disease References

Infantile Refsum's disease
Poll-The BT, Saudubray JM, Ogier H, Schutgens RB, Wanders RJ, Schrakamp G, van den Bosch H, Trijbels JM, Poulos A, Moser HW, et al.: Infantile Refsum's disease: biochemical findings suggesting multiple peroxisomal dysfunction. J Inherit Metab Dis. 1986;9(2):169-74. [PubMed:2427795 ] Poulos A, Sharp P, Fellenberg AJ, Danks DM: Cerebro-hepato-renal (Zellweger) syndrome, adrenoleukodystrophy, and Refsum's disease: plasma changes and skin fibroblast phytanic acid oxidase. Hum Genet. 1985;70(2):172-7. [PubMed:2408988 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Rhizomelic chondrodysplasia punctata
Baumgartner MR, Poll-The BT, Verhoeven NM, Jakobs C, Espeel M, Roels F, Rabier D, Levade T, Rolland MO, Martinez M, Wanders RJ, Saudubray JM: Clinical approach to inherited peroxisomal disorders: a series of 27 patients. Ann Neurol. 1998 Nov;44(5):720-30. [PubMed:9818927 ]
Refsum's disease
Poulos A, Sharp P, Fellenberg AJ, Danks DM: Cerebro-hepato-renal (Zellweger) syndrome, adrenoleukodystrophy, and Refsum's disease: plasma changes and skin fibroblast phytanic acid oxidase. Hum Genet. 1985;70(2):172-7. [PubMed:2408988 ]
Peroxisomal disorders, new type, liver
Mandel H, Espeel M, Roels F, Sofer N, Luder A, Iancu TC, Aizin A, Berant M, Wanders RJ, Schutgens RB: A new type of peroxisomal disorder with variable expression in liver and fibroblasts. J Pediatr. 1994 Oct;125(4):549-55. [PubMed:7931872 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Pyridoxine-dependent epilepsy
Plecko B, Paul K, Paschke E, Stoeckler-Ipsiroglu S, Struys E, Jakobs C, Hartmann H, Luecke T, di Capua M, Korenke C, Hikel C, Reutershahn E, Freilinger M, Baumeister F, Bosch F, Erwa W: Biochemical and molecular characterization of 18 patients with pyridoxine-dependent epilepsy and mutations of the antiquitin (ALDH7A1) gene. Hum Mutat. 2007 Jan;28(1):19-26. [PubMed:17068770 ]
D-Bifunctional protein deficiency
Watkins PA, Chen WW, Harris CJ, Hoefler G, Hoefler S, Blake DC Jr, Balfe A, Kelley RI, Moser AB, Beard ME, et al.: Peroxisomal bifunctional enzyme deficiency. J Clin Invest. 1989 Mar;83(3):771-7. [PubMed:2921319 ]
Pseudoneonatal adrenoleukodystrophy
Poll-The BT, Roels F, Ogier H, Scotto J, Vamecq J, Schutgens RB, Wanders RJ, van Roermund CW, van Wijland MJ, Schram AW, et al.: A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo-neonatal adrenoleukodystrophy). Am J Hum Genet. 1988 Mar;42(3):422-34. [PubMed:2894756 ]
Traumatic brain injury
Smythe GA, Poljak A, Bustamante S, Braga O, Maxwell A, Grant R, Sachdev P: ECNI GC-MS analysis of picolinic and quinolinic acids and their amides in human plasma, CSF, and brain tissue. Adv Exp Med Biol. 2003;527:705-12. [PubMed:15206793 ]
Malaria
Medana IM, Hien TT, Day NP, Phu NH, Mai NT, Chu'ong LV, Chau TT, Taylor A, Salahifar H, Stocker R, Smythe G, Turner GD, Farrar J, White NJ, Hunt NH: The clinical significance of cerebrospinal fluid levels of kynurenine pathway metabolites and lactate in severe malaria. J Infect Dis. 2002 Mar 1;185(5):650-6. Epub 2002 Feb 14. [PubMed:11865422 ] Medana IM, Day NP, Salahifar-Sabet H, Stocker R, Smythe G, Bwanaisa L, Njobvu A, Kayira K, Turner GD, Taylor TE, Hunt NH: Metabolites of the kynurenine pathway of tryptophan metabolism in the cerebrospinal fluid of Malawian children with malaria. J Infect Dis. 2003 Sep 15;188(6):844-9. Epub 2003 Sep 9. [PubMed:12964115 ]
Anemia
Medana IM, Day NP, Salahifar-Sabet H, Stocker R, Smythe G, Bwanaisa L, Njobvu A, Kayira K, Turner GD, Taylor TE, Hunt NH: Metabolites of the kynurenine pathway of tryptophan metabolism in the cerebrospinal fluid of Malawian children with malaria. J Infect Dis. 2003 Sep 15;188(6):844-9. Epub 2003 Sep 9. [PubMed:12964115 ]
Tuberculous meningitis
Medana IM, Day NP, Salahifar-Sabet H, Stocker R, Smythe G, Bwanaisa L, Njobvu A, Kayira K, Turner GD, Taylor TE, Hunt NH: Metabolites of the kynurenine pathway of tryptophan metabolism in the cerebrospinal fluid of Malawian children with malaria. J Infect Dis. 2003 Sep 15;188(6):844-9. Epub 2003 Sep 9. [PubMed:12964115 ]
Convulsion
Medana IM, Day NP, Salahifar-Sabet H, Stocker R, Smythe G, Bwanaisa L, Njobvu A, Kayira K, Turner GD, Taylor TE, Hunt NH: Metabolites of the kynurenine pathway of tryptophan metabolism in the cerebrospinal fluid of Malawian children with malaria. J Infect Dis. 2003 Sep 15;188(6):844-9. Epub 2003 Sep 9. [PubMed:12964115 ]
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 ]
Hyperpipecolatemia
Gatfield PD, Taller E, Hinton GG, Wallace AC, Abdelnour GM, Haust MD: Hyperpipecolatemia: A new metabolic disorder associated with neuropathy and hepatomegaly: A case study. Can Med Assoc J. 1968 Dec 28;99(25):1215-33. [PubMed:5700850 ]
Colorectal cancer
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 ]
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 ]
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 ]
Peroxisomal biogenesis defect
van den Berg GA, Breukelman H, Elzinga H, Trijbels JM, Monnens LA, Muskiet FA: Determination of pipecolic acid in urine and plasma by isotope dilution mass fragmentography. Clin Chim Acta. 1986 Sep 30;159(3):229-37. [PubMed:3769211 ] Baas JC, van de Laar R, Dorland L, Duran M, Berger R, Poll-The BT, de Koning TJ: Plasma pipecolic acid is frequently elevated in non-peroxisomal disease. J Inherit Metab Dis. 2002 Dec;25(8):699-701. [PubMed:12705501 ] Fujita T, Hada T, Higashino K: Origin of D- and L-pipecolic acid in human physiological fluids: a study of the catabolic mechanism to pipecolic acid using the lysine loading test. Clin Chim Acta. 1999 Sep;287(1-2):145-56. [PubMed:10509903 ] Baumgartner MR, Poll-The BT, Verhoeven NM, Jakobs C, Espeel M, Roels F, Rabier D, Levade T, Rolland MO, Martinez M, Wanders RJ, Saudubray JM: Clinical approach to inherited peroxisomal disorders: a series of 27 patients. Ann Neurol. 1998 Nov;44(5):720-30. [PubMed:9818927 ] Budden SS, Kennaway NG, Buist NR, Poulos A, Weleber RG: Dysmorphic syndrome with phytanic acid oxidase deficiency, abnormal very long chain fatty acids, and pipecolic acidemia: studies in four children. J Pediatr. 1986 Jan;108(1):33-9. [PubMed:2418187 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Adrenoleukodystrophy
Poulos A, Sharp P, Fellenberg AJ, Danks DM: Cerebro-hepato-renal (Zellweger) syndrome, adrenoleukodystrophy, and Refsum's disease: plasma changes and skin fibroblast phytanic acid oxidase. Hum Genet. 1985;70(2):172-7. [PubMed:2408988 ] G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Schizophrenia
Yang J, Chen T, Sun L, Zhao Z, Qi X, Zhou K, Cao Y, Wang X, Qiu Y, Su M, Zhao A, Wang P, Yang P, Wu J, Feng G, He L, Jia W, Wan C: Potential metabolite markers of schizophrenia. Mol Psychiatry. 2013 Jan;18(1):67-78. doi: 10.1038/mp.2011.131. Epub 2011 Oct 25. [PubMed:22024767 ] Al Awam K, Haussleiter IS, Dudley E, Donev R, Brune M, Juckel G, Thome J: Multiplatform metabolome and proteome profiling identifies serum metabolite and protein signatures as prospective biomarkers for schizophrenia. J Neural Transm (Vienna). 2015 Aug;122 Suppl 1:S111-22. doi: 10.1007/s00702-014-1224-0. Epub 2014 May 1. [PubMed:24789758 ]

Associated OMIM IDs

266510 (Infantile Refsum's disease)
215100 (Rhizomelic chondrodysplasia punctata)
266500 (Refsum's disease)
266100 (Pyridoxine-dependent epilepsy)
261515 (D-Bifunctional protein deficiency)
264470 (Pseudoneonatal adrenoleukodystrophy)
248310 (Malaria)
238700 (Hyperlysinemia I, familial)
114500 (Colorectal cancer)
260350 (Pancreatic cancer)
170650 (Periodontal disease)
214100 (Peroxisomal biogenesis defect)
300100 (Adrenoleukodystrophy)
181500 (Schizophrenia)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

KNApSAcK ID

Chemspider ID

KEGG Compound ID

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Pipecolic_acid

METLIN ID

PubChem Compound

849

PDB ID

Not Available

ChEBI ID

17964

Food Biomarker Ontology

Not Available

VMH ID

Not Available

MarkerDB ID

MDB00000036

Good Scents ID

rw1586071

References

Synthesis Reference

Asher, Vikram; Becu, Christian; Anteunis, Marc J. O.; Callens, Roland. New synthesis of pipecolic acid and analogs. Tetrahedron Letters (1981), 22(2), 141-4.

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 ]
Kawasaki H, Hori T, Nakajima M, Takeshita K: Plasma levels of pipecolic acid in patients with chronic liver disease. Hepatology. 1988 Mar-Apr;8(2):286-9. [PubMed:3356409 ]
Chang YF: Lysine metabolism in the human and the monkey: demonstration of pipecolic acid formation in the brain and other organs. Neurochem Res. 1982 May;7(5):577-88. [PubMed:6811962 ]
van den Berg GA, Breukelman H, Elzinga H, Trijbels JM, Monnens LA, Muskiet FA: Determination of pipecolic acid in urine and plasma by isotope dilution mass fragmentography. Clin Chim Acta. 1986 Sep 30;159(3):229-37. [PubMed:3769211 ]
Medana IM, Hien TT, Day NP, Phu NH, Mai NT, Chu'ong LV, Chau TT, Taylor A, Salahifar H, Stocker R, Smythe G, Turner GD, Farrar J, White NJ, Hunt NH: The clinical significance of cerebrospinal fluid levels of kynurenine pathway metabolites and lactate in severe malaria. J Infect Dis. 2002 Mar 1;185(5):650-6. Epub 2002 Feb 14. [PubMed:11865422 ]
Danks DM, Tippett P, Adams C, Campbell P: Cerebro-hepato-renal syndrome of Zellweger. A report of eight cases with comments upon the incidence, the liver lesion, and a fault in pipecolic acid metabolism. J Pediatr. 1975 Mar;86(3):382-7. [PubMed:1113225 ]
Plecko B, Stockler-Ipsiroglu S, Paschke E, Erwa W, Struys EA, Jakobs C: Pipecolic acid elevation in plasma and cerebrospinal fluid of two patients with pyridoxine-dependent epilepsy. Ann Neurol. 2000 Jul;48(1):121-5. [PubMed:10894227 ]
Burton BK, Reed SP, Remy WT: Hyperpipecolic acidemia: clinical and biochemical observations in two male siblings. J Pediatr. 1981 Nov;99(5):729-34. [PubMed:7299546 ]
Smythe GA, Poljak A, Bustamante S, Braga O, Maxwell A, Grant R, Sachdev P: ECNI GC-MS analysis of picolinic and quinolinic acids and their amides in human plasma, CSF, and brain tissue. Adv Exp Med Biol. 2003;527:705-12. [PubMed:15206793 ]
Trijbels JM, Monnens LA, Melis G, van den Broekvan Essen M, Bruckwilder M: Localization of pipecolic acid metabolism in rat liver peroxisomes: probable explanation for hyperpipecolataemia in Zellweger syndrome. J Inherit Metab Dis. 1987;10(2):128-34. [PubMed:3116331 ]
Thomas GH, Haslam RH, Batshaw ML, Capute AJ, Neidengard L, Ransom JL: Hyperpipecolic acidemia associated with hepatomegaly, mental retardation, optic nerve dysplasia and progressive neurological disease. Clin Genet. 1975 Nov;8(5):376-82. [PubMed:1204235 ]
Medana IM, Day NP, Salahifar-Sabet H, Stocker R, Smythe G, Bwanaisa L, Njobvu A, Kayira K, Turner GD, Taylor TE, Hunt NH: Metabolites of the kynurenine pathway of tryptophan metabolism in the cerebrospinal fluid of Malawian children with malaria. J Infect Dis. 2003 Sep 15;188(6):844-9. Epub 2003 Sep 9. [PubMed:12964115 ]
Lee HF, Mak SC, Wu FW, Chi CS, Huang SC: Zellweger syndrome: report of one case. Acta Paediatr Taiwan. 2001 Jan-Feb;42(1):53-6. [PubMed:11270189 ]
Kok RM, Kaster L, de Jong AP, Poll-The B, Saudubray JM, Jakobs C: Stable isotope dilution analysis of pipecolic acid in cerebrospinal fluid, plasma, urine and amniotic fluid using electron capture negative ion mass fragmentography. Clin Chim Acta. 1987 Sep 30;168(2):143-52. [PubMed:3315316 ]
Baas JC, van de Laar R, Dorland L, Duran M, Berger R, Poll-The BT, de Koning TJ: Plasma pipecolic acid is frequently elevated in non-peroxisomal disease. J Inherit Metab Dis. 2002 Dec;25(8):699-701. [PubMed:12705501 ]
Dancis J, Hutzler J: Comparative rates of metabolism of pipecolic acid in several animal species. Comp Biochem Physiol B. 1982;73(4):1011-2. [PubMed:6817963 ]
Fujita T, Hada T, Higashino K: Origin of D- and L-pipecolic acid in human physiological fluids: a study of the catabolic mechanism to pipecolic acid using the lysine loading test. Clin Chim Acta. 1999 Sep;287(1-2):145-56. [PubMed:10509903 ]
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 ]

Enzymes

Enzyme Details

1. Peroxisomal sarcosine oxidase

General function:: Involved in oxidoreductase activity
Specific function:: Metabolizes sarcosine, L-pipecolic acid and L-proline.
Gene Name:: PIPOX
Uniprot ID:: Q9P0Z9
Molecular weight:: 44065.515

Showing metabocard for Pipecolic acid (HMDB0000070)

MOL for HMDB0000070 (Pipecolic acid)

3D MOL for HMDB0000070 (Pipecolic acid)

3D SDF for HMDB0000070 (Pipecolic acid)

3D-SDF for HMDB0000070 (Pipecolic acid)

PDB for HMDB0000070 (Pipecolic acid)

3D PDB for HMDB0000070 (Pipecolic acid)

SMILES for HMDB0000070 (Pipecolic acid)

INCHI for HMDB0000070 (Pipecolic acid)

Structure for HMDB0000070 (Pipecolic acid)

3D Structure for HMDB0000070 (Pipecolic acid)

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra

IR Spectra

Enzymes