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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-12-07 01:19:28 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
Thumb
SynonymsNot Available
Chemical FormulaC6H11NO2
Average Molecular Weight129.157
Monoisotopic Molecular Weight129.078978601
IUPAC NameNot Available
Traditional NameNot Available
CAS Registry Number3105-95-1
SMILESNot Available
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
ClassificationNot classified
Ontology
Physiological effect

Health effect:

  Health condition:

Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

  Organ and components:

Source:

Process

Naturally occurring process:

  Biological process:

    Biochemical pathway:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point272 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted PropertiesNot Available
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0a4i-0900000000-c7875e682dfb275cdf1eView in MoNA
GC-MSGC-MS Spectrum - GC-MS (1 TMS)splash10-001i-9100000000-07741b1b7d650447197eView in MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-0a4i-1900000000-1b1313072749567d8a9eView in MoNA
GC-MSGC-MS Spectrum - GC-MS (1 MEOX; 2 TMS)splash10-0pb9-3960000000-8e89c177d71b5faabdb2View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0a4i-0900000000-c7875e682dfb275cdf1eView in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0a4i-1900000000-1b1313072749567d8a9eView in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0pb9-3960000000-8e89c177d71b5faabdb2View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-001i-9100000000-07741b1b7d650447197eView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-001i-9100000000-335cf7f41fca5cbc84cfView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-05fu-9700000000-8003521353d4d37af49fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, N/A (Annotated)splash10-001i-8900000000-b67eb6eabd8fb1b2824aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, N/A (Annotated)splash10-001i-9000000000-b01a5574139ce1222c4dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, N/A (Annotated)splash10-0a4i-9000000000-d80733b289711920626bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-004i-0900000000-6d82525e15d98dd794dbView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-004i-0900000000-abb8b4f2daaa91bfa758View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-004i-1900000000-b668d1df137fa1685b6dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-0002-9000000000-5ec763c9c55715bd01dfView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0002-9000000000-5a62167bd787768808f7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-001i-0900000000-edd41fa2c224ec5f5a1eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-001i-9100000000-7ed3d7197cd0f23403b6View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-001i-9000000000-e5b2220b7410a3429b95View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-001i-9000000000-baf8dab4263881ac49eaView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-0a59-9000000000-d1dc7829582bfd61d47bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-004i-0900000000-6d82525e15d98dd794dbView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-004i-0900000000-abb8b4f2daaa91bfa758View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-004i-1900000000-b668d1df137fa1685b6dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0002-9000000000-5ec763c9c55715bd01dfView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0002-9000000000-5a62167bd787768808f7View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-001i-0900000000-edd41fa2c224ec5f5a1eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-001i-3900000000-bf71d20a7c8e71d335e5View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-001i-9400000000-fa70645b026293d659deView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-053u-9000000000-f849cc3c4b11bc7d4de7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-004i-2900000000-bf800b5cd197887a11d5View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-003r-7900000000-8a0e9f51df4006740863View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-001i-9000000000-effc196b5c15971f985aView in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Peroxisome
Biofluid Locations
  • Blood
  • Feces
Tissue Location
  • Liver
Pathways
NameSMPDB/PathwhizKEGG
2-aminoadipic 2-oxoadipic aciduriaPw000696Pw000696 greyscalePw000696 simpleNot Available
Glutaric Aciduria Type IPw000111Pw000111 greyscalePw000111 simpleNot Available
Hyperlysinemia I, FamilialPw000503Pw000503 greyscalePw000503 simpleNot Available
Hyperlysinemia II or SaccharopinuriaPw000504Pw000504 greyscalePw000504 simpleNot Available
Lysine DegradationPw000029Pw000029 greyscalePw000029 simpleMap00310
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
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 Quantified Not SpecifiedNot Specified
Normal
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
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
Associated Disorders and Diseases
Disease References
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 ]
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 ]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB000546
KNApSAcK IDNot Available
Chemspider ID388365
KEGG Compound IDC00408
BioCyc IDL-PIPECOLATE
BiGG ID34890
Wikipedia LinkNot Available
METLIN ID5684
PubChem Compound439227
PDB IDYCP
ChEBI ID30913
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 → (S)-2,3,4,5-tetrahydropyridine-2-carboxylate + Hydrogen peroxidedetails