Hmdb loader
You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2022-09-22 17:43:44 UTC
HMDB IDHMDB0000716
Secondary Accession Numbers
  • HMDB00716
Metabolite Identification
Common NameL-Pipecolic acid
DescriptionL-pipecolic acid is a normal human metabolite present in human blood, where is present as the primary enantiomer of pipecolic acid. L-pipecolic acid is a cyclic imino acid (contains both imino (>C=NH) and carboxyl (-C(=O)-OH) functional groups) produced during the degradation of lysine, accumulates in body fluids of infants with generalized genetic peroxisomal disorders, including Zellweger syndrome (OMIM 214100 ), neonatal adrenoleukodystrophy (OMIM 202370 ), and infantile Refsum disease (OMIM 266510 ). L-pipecolic acid levels are also elevated in patients with chronic liver diseases. L-pipecolic acid is the substrate of delta1-piperideine-2-carboxylate reductase (EC 1.5.1.21) in the pathway of lysine degradation (PMID: 2717271 , 8305590 , 1050990 ).
Structure
Data?1582752151
Synonyms
ValueSource
(S)-(-)-Pipecolic acidChEBI
(S)-Pipecolic acidChEBI
(S)-Piperidine-2-carboxylic acidChEBI
2-Piperidinecarboxylic acidChEBI
L-(-)-Pipecolic acidChEBI
Pipecolic acidChEBI
Pipecolinic acidChEBI
(S)-(-)-PipecolateGenerator
(S)-PipecolateGenerator
(S)-Piperidine-2-carboxylateGenerator
2-PiperidinecarboxylateGenerator
L-(-)-PipecolateGenerator
PipecolateGenerator
PipecolinateGenerator
L-PipecolateGenerator
(-)-PipecolateHMDB
(-)-Pipecolic acidHMDB
(S)-(-)-2-PiperidinecarboxylateHMDB
(S)-(-)-2-Piperidinecarboxylic acidHMDB
(S)-2-PiperidinecarboxylateHMDB
(S)-2-Piperidinecarboxylic acidHMDB
(S)-PipecolinateHMDB
(S)-Pipecolinic acidHMDB
L-HomoprolineHMDB
L-PipecolinateHMDB
L-Pipecolinic acidHMDB
L-Piperidine-2-carboxylateHMDB
L-Piperidine-2-carboxylic acidHMDB
Pipecolic acid, (+,-)-isomerHMDB
Pipecolic acid, 14C-labeled CPD, (+,-)-isomerHMDB
Homopipecolic acidHMDB
Pipecolic acid, ion(1-), (+,-)-isomerHMDB
Pipecolic acid, ion(1-), (S)-isomerHMDB
Pipecolic acid hydrochloride, (+-)-isomerHMDB
Pipecolic acid, (S)-isomerHMDB
Pipecolic acid, (R)-isomerHMDB
Pipecolic acid, ion (1-)HMDB
Pipecolic acid, monopotassium saltHMDB
(2S)-2-Piperidinecarboxylic acidHMDB
(-)-Pipecolinic acidHMDB
(2S)-Piperidine-2-carboxylic acidHMDB
(RS)-2-Piperidinecarboxylic acidHMDB
(±)-2-piperidinecarboxylic acidHMDB
(±)-pipecolic acidHMDB
(±)-pipecolinic acidHMDB
2-CarboxypiperidineHMDB
2-Pipecolinic acidHMDB
2-Piperidinylcarboxylic acidHMDB
DL-2-Piperidinecarboxylic acidHMDB
DL-Pipecolic acidHMDB
DL-Pipecolinic acidHMDB
DihydrobaikianeHMDB
Hexahydro-2-picolinic acidHMDB
HomoprolineHMDB
Piperidine-6-carboxylic acidHMDB
Piperolinic acidHMDB
alpha-Pipecolinic acidHMDB
Α-pipecolinic acidHMDB
L-Pipecolic acidPhytoBank
Chemical FormulaC6H11NO2
Average Molecular Weight129.157
Monoisotopic Molecular Weight129.078978601
IUPAC Name(2S)-piperidine-2-carboxylic acid
Traditional NameL-pipecolic acid
CAS Registry Number3105-95-1
SMILES
OC(=O)[C@@H]1CCCCN1
InChI Identifier
InChI=1S/C6H11NO2/c8-6(9)5-3-1-2-4-7-5/h5,7H,1-4H2,(H,8,9)/t5-/m0/s1
InChI KeyHXEACLLIILLPRG-YFKPBYRVSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as l-alpha-amino acids. These are alpha amino acids which have the L-configuration of the alpha-carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentL-alpha-amino acids
Alternative Parents
Substituents
  • L-alpha-amino acid
  • Piperidinecarboxylic acid
  • Piperidine
  • Amino acid
  • Carboxylic acid
  • Secondary aliphatic amine
  • Monocarboxylic acid or derivatives
  • Secondary amine
  • Organoheterocyclic compound
  • Azacycle
  • Organic oxygen compound
  • Organic nitrogen compound
  • Organooxygen compound
  • Organonitrogen compound
  • Amine
  • Carbonyl group
  • Organopnictogen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Aliphatic heteromonocyclic compound
Molecular FrameworkAliphatic heteromonocyclic compounds
External Descriptors
Ontology
Disposition

Biological location

Source

Process

Naturally occurring process

Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point272 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility74060 mg/L @ 25 °C (est)The Good Scents Company Information System
LogPNot AvailableNot Available
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Baker133.81530932474
[M-H]-MetCCS_train_neg126.71330932474
[M+H]+Baker130.93330932474
[M+H]+MetCCS_train_pos127.40230932474
[M-H]-Not Available128.6http://allccs.zhulab.cn/database/detail?ID=AllCCS00000028
[M+H]+Not Available128.4http://allccs.zhulab.cn/database/detail?ID=AllCCS00000028
Predicted Molecular Properties
PropertyValueSource
Water Solubility158 g/LALOGPS
logP-2.2ALOGPS
logP-2.1ChemAxon
logS0.09ALOGPS
pKa (Strongest Acidic)2.06ChemAxon
pKa (Strongest Basic)10.39ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area49.33 ŲChemAxon
Rotatable Bond Count1ChemAxon
Refractivity32.67 m³·mol⁻¹ChemAxon
Polarizability13.49 ųChemAxon
Number of Rings1ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+127.9531661259
DarkChem[M-H]-121.60231661259
AllCCS[M+H]+127.91432859911
AllCCS[M-H]-125.3132859911
DeepCCS[M+H]+126.23630932474
DeepCCS[M-H]-122.76730932474
DeepCCS[M-2H]-159.78130932474
DeepCCS[M+Na]+135.31930932474

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
L-Pipecolic acidOC(=O)[C@@H]1CCCCN11900.4Standard polar33892256
L-Pipecolic acidOC(=O)[C@@H]1CCCCN11157.5Standard non polar33892256
L-Pipecolic acidOC(=O)[C@@H]1CCCCN11246.2Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
L-Pipecolic acid,1TMS,isomer #1C[Si](C)(C)OC(=O)[C@@H]1CCCCN11261.2Semi standard non polar33892256
L-Pipecolic acid,1TMS,isomer #2C[Si](C)(C)N1CCCC[C@H]1C(=O)O1355.4Semi standard non polar33892256
L-Pipecolic acid,2TMS,isomer #1C[Si](C)(C)OC(=O)[C@@H]1CCCCN1[Si](C)(C)C1344.3Semi standard non polar33892256
L-Pipecolic acid,2TMS,isomer #1C[Si](C)(C)OC(=O)[C@@H]1CCCCN1[Si](C)(C)C1406.9Standard non polar33892256
L-Pipecolic acid,2TMS,isomer #1C[Si](C)(C)OC(=O)[C@@H]1CCCCN1[Si](C)(C)C1703.7Standard polar33892256
L-Pipecolic acid,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)[C@@H]1CCCCN11500.1Semi standard non polar33892256
L-Pipecolic acid,1TBDMS,isomer #2CC(C)(C)[Si](C)(C)N1CCCC[C@H]1C(=O)O1575.4Semi standard non polar33892256
L-Pipecolic acid,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)[C@@H]1CCCCN1[Si](C)(C)C(C)(C)C1828.0Semi standard non polar33892256
L-Pipecolic acid,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)[C@@H]1CCCCN1[Si](C)(C)C(C)(C)C1828.8Standard non polar33892256
L-Pipecolic acid,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)[C@@H]1CCCCN1[Si](C)(C)C(C)(C)C1961.8Standard polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental GC-MSGC-MS Spectrum - L-Pipecolic acid GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0a4i-0900000000-c7875e682dfb275cdf1e2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Pipecolic acid GC-MS (1 TMS)splash10-001i-9100000000-07741b1b7d650447197e2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Pipecolic acid GC-MS (2 TMS)splash10-0a4i-1900000000-1b1313072749567d8a9e2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Pipecolic acid GC-MS (1 MEOX; 2 TMS)splash10-0pb9-3960000000-8e89c177d71b5faabdb22014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Pipecolic acid GC-EI-TOF (Non-derivatized)splash10-0a4i-0900000000-c7875e682dfb275cdf1e2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Pipecolic acid GC-MS (Non-derivatized)splash10-0a4i-1900000000-1b1313072749567d8a9e2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Pipecolic acid GC-MS (Non-derivatized)splash10-0pb9-3960000000-8e89c177d71b5faabdb22017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - L-Pipecolic acid GC-MS (Non-derivatized)splash10-001i-9100000000-07741b1b7d650447197e2017-09-12HMDB team, MONA, MassBankView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Pipecolic acid GC-MS (Non-derivatized) - 70eV, Positivesplash10-001i-9100000000-335cf7f41fca5cbc84cf2017-09-01Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Pipecolic acid GC-MS (1 TMS) - 70eV, Positivesplash10-05fu-9700000000-8003521353d4d37af49f2017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Pipecolic acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Pipecolic acid GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Pipecolic acid GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - L-Pipecolic acid GC-MS (TBDMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid Quattro_QQQ 10V, N/A-QTOF (Annotated)splash10-001i-8900000000-b67eb6eabd8fb1b2824a2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid Quattro_QQQ 25V, N/A-QTOF (Annotated)splash10-001i-9000000000-b01a5574139ce1222c4d2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid Quattro_QQQ 40V, N/A-QTOF (Annotated)splash10-0a4i-9000000000-d80733b289711920626b2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative-QTOFsplash10-004i-0900000000-6d82525e15d98dd794db2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative-QTOFsplash10-004i-0900000000-abb8b4f2daaa91bfa7582012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative-QTOFsplash10-004i-1900000000-b668d1df137fa1685b6d2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative-QTOFsplash10-0002-9000000000-5ec763c9c55715bd01df2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative-QTOFsplash10-0002-9000000000-5a62167bd787768808f72012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ , negative-QTOFsplash10-004i-0900000000-6d82525e15d98dd794db2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ , negative-QTOFsplash10-004i-0900000000-abb8b4f2daaa91bfa7582017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ , negative-QTOFsplash10-004i-1900000000-b668d1df137fa1685b6d2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ , negative-QTOFsplash10-0002-9000000000-5ec763c9c55715bd01df2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ , negative-QTOFsplash10-0002-9000000000-5a62167bd787768808f72017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid 20V, Negative-QTOFsplash10-001i-9000000000-ead57aa6faae96fa4d3b2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid 35V, Negative-QTOFsplash10-004i-0900000000-19b8ea0a6fe6e8c38e152021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid 10V, Negative-QTOFsplash10-004i-0900000000-2dae6edf7d6d60c927242021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid 35V, Negative-QTOFsplash10-004i-0900000000-361ed03db21e35e783832021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positive-QTOFsplash10-001i-0900000000-edd41fa2c224ec5f5a1e2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positive-QTOFsplash10-001i-9100000000-7ed3d7197cd0f23403b62012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positive-QTOFsplash10-001i-9000000000-e5b2220b7410a3429b952012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positive-QTOFsplash10-001i-9000000000-baf8dab4263881ac49ea2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positive-QTOFsplash10-0a59-9000000000-d1dc7829582bfd61d47b2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ , positive-QTOFsplash10-001i-0900000000-edd41fa2c224ec5f5a1e2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ , positive-QTOFsplash10-001i-9100000000-a4a80ec7473a698297952017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - L-Pipecolic acid LC-ESI-QQ , positive-QTOFsplash10-001i-9000000000-e5b2220b7410a3429b952017-09-14HMDB team, MONAView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Experimental 1D NMR1H NMR Spectrum (1D, 600 MHz, H2O, experimental)2012-12-04Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-05Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Peroxisome
Biospecimen Locations
  • Blood
  • Feces
  • Urine
Tissue Locations
  • Liver
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified10.0 (0.0-20.0) uMChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified2.46 +/- 1.26 uMAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified9.7 +/- 3.1 uMAdult (>18 years old)BothZellweger syndrome details
BloodDetected and Quantified65.0 +/- 63.0 uMNewborn (0-30 days old)BothAdrenoleukodystrophy (ALD) details
UrineDetected but not QuantifiedNot QuantifiedNot SpecifiedNot SpecifiedCancer patients undergoing total body irradiation details
Associated Disorders and Diseases
Disease References
Peroxisomal biogenesis defect
  1. 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 ]
Adrenoleukodystrophy
  1. 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 ]
Associated OMIM IDs
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB000546
KNApSAcK IDC00001387
Chemspider ID388365
KEGG Compound IDC00408
BioCyc IDL-PIPECOLATE
BiGG ID34890
Wikipedia LinkPipecolic acid
METLIN ID5684
PubChem Compound439227
PDB IDNot Available
ChEBI ID30913
Food Biomarker OntologyNot Available
VMH IDLPIPECOL
MarkerDB IDMDB00000233
Good Scents IDrw1253961
References
Synthesis ReferenceKisfaludy, Lajos; Korenczki, Ferenc; Katho, Agnes. Works, Gedeon Richter Ltd., Budapest, Hung. One-step synthesis of L-piperidine-2-carboxylic acid. Synthesis (1982), (2), 163.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Mihalik SJ, Moser HW, Watkins PA, Danks DM, Poulos A, Rhead WJ: Peroxisomal L-pipecolic acid oxidation is deficient in liver from Zellweger syndrome patients. Pediatr Res. 1989 May;25(5):548-52. [PubMed:2717271 ]
  2. Baumgartner MR, Jansen GA, Verhoeven NM, Mooyer PA, Jakobs C, Roels F, Espeel M, Fourmaintraux A, Bellet H, Wanders RJ, Saudubray JM: Atypical refsum disease with pipecolic acidemia and abnormal catalase distribution. Ann Neurol. 2000 Jan;47(1):109-13. [PubMed:10632109 ]
  3. Lam S: Stereoselective analysis of D and L dansyl amino acids as the mixed chelate copper(II) complexes by HPLC. J Chromatogr Sci. 1984 Sep;22(9):416-23. [PubMed:6490790 ]
  4. Armstrong DW, Zukowski J, Ercal N, Gasper M: Stereochemistry of pipecolic acid found in the urine and plasma of subjects with peroxisomal deficiencies. J Pharm Biomed Anal. 1993 Oct;11(10):881-6. [PubMed:8305590 ]
  5. Langeland R: [Construction of facilities in the health sector]. Sykepleien. 1976 Sep 5;63(15):782-4, 793. [PubMed:1050990 ]

Enzymes

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
Reactions
L-Pipecolic acid + Oxygen → 2,3,4,5-Tetrahydro-2-pyridinecarboxylic acid + Hydrogen peroxidedetails