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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-10-19 22:10:33 UTC
HMDB IDHMDB00070
Secondary Accession NumbersNone
Metabolite Identification
Common NamePipecolic acid
DescriptionPipecolic 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 ).
Structure
Thumb
Synonyms
ValueSource
2-Piperidinecarboxylic acidChEBI
HomoprolineChEBI
Pipecolinic acidChEBI
2-PiperidinecarboxylateGenerator
Piperidine-2-carboxylateGenerator
PipecolinateGenerator
()-Piperidine-2-carboxylic acidHMDB
(+/-)-2-piperidinecarboxylateHMDB
(+/-)-2-piperidinecarboxylic acidHMDB
(+/-)-pipecolateHMDB
(+/-)-pipecolic acidHMDB
(+/-)-pipecolinateHMDB
(+/-)-pipecolinic acidHMDB
(.+/-.)-2-piperidinecarboxylic acidHMDB
(RS)-2-PiperidinecarboxylateHMDB
(RS)-2-Piperidinecarboxylic acidHMDB
.alpha.-pipecolinic acidHMDB
2-CarboxypiperidineHMDB
2-Pipecolinic acidHMDB
2-Piperidinylcarboxylic acidHMDB
a-PipecolinateHMDB
a-Pipecolinic acidHMDB
Acide pipecoliqueHMDB
Acide piperidine-carboxylique-2HMDB
alpha-PipecolinateHMDB
alpha-Pipecolinic acidHMDB
DihydrobaikianeHMDB
DL-2-PiperidinecarboxylateHMDB
DL-2-Piperidinecarboxylic acidHMDB
DL-HomoprolineHMDB
DL-PipecolateHMDB
DL-Pipecolic acidHMDB
DL-PipecolinateHMDB
DL-Pipecolinic acidHMDB
hexahydro-2-PicolinateHMDB
hexahydro-2-Picolinic acidHMDB
HexahydropicolinateHMDB
Hexahydropicolinic acidHMDB
PipecolateHMDB
Pipecolic acid free baseHMDB
PiperolinateHMDB
Piperolinic acidHMDB
Chemical FormulaC6H11NO2
Average Molecular Weight129.157
Monoisotopic Molecular Weight129.078978601
IUPAC Namepiperidine-2-carboxylic acid
Traditional Name(+,-)-pipecolic acid
CAS Registry Number535-75-1
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 KeyInChIKey=HXEACLLIILLPRG-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound 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).
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentAlpha amino acids
Alternative Parents
Substituents
  • Alpha-amino acid
  • Piperidinecarboxylic acid
  • Piperidine
  • Azacycle
  • Organoheterocyclic compound
  • Secondary amine
  • Monocarboxylic acid or derivatives
  • Secondary aliphatic amine
  • Carboxylic acid
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Amine
  • Aliphatic heteromonocyclic compound
Molecular FrameworkAliphatic heteromonocyclic compounds
External DescriptorsNot Available
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
  • Microbial
Biofunction
  • Protein synthesis, amino acid biosynthesis
ApplicationNot Available
Cellular locations
  • Cytoplasm
  • Extracellular
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point264 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility314 mg/mLNot Available
LogP-2.31TSAI,RS ET AL. (1991)
Predicted Properties
PropertyValueSource
Water Solubility158.0 mg/mLALOGPS
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 Å2ChemAxon
Rotatable Bond Count1ChemAxon
Refractivity32.67 m3·mol-1ChemAxon
Polarizability13.47 Å3ChemAxon
Number of Rings1ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MSNot Available
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0060-9900000000-c5b5bf28424d5d5d1bdaView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-001i-9000000000-92660ad26bff36fba7efView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0a59-9000000000-b779372fa816467754c1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positivesplash10-001i-9300000000-26dee47121401990f1c1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) 30V, Positivesplash10-001i-9000000000-929057dfd61546bee24dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-004i-0900000000-6595801dc6a322f712f0View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-001i-3900000000-b2ceb4c88f50a6524604View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-001i-9400000000-d3fab76e3fbfbe92c14dView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a5c-9000000000-20464a5c62992da01ab7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-004i-2900000000-6fac2bf48ffe4d910118View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-003r-8900000000-81b6d69c7a568e36b49fView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-001i-9000000000-6332d1986eb0da0057fdView in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,1H] 2D NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
Biofluid Locations
  • Amniotic Fluid
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Location
  • Gut
  • Liver
  • Prostate
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
Amniotic FluidDetected and Quantified4.65 (2.24 - 8.40) uMNot SpecifiedBothNormal details
BloodDetected and Quantified2.46 +/- 1.26 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0-5 uMNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified0-5 uMInfant (1 - 3 months old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified5.73 (3.75 - 10.8) uMNewborn (0-30 days old)Both
Normal
details
BloodDetected and Quantified0-10 uMChildren (3 months - 6 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified0-10 uMChildren (6 - 18 years old)Both
Normal
    • BC Children's Hos...
details
BloodDetected and Quantified1.46 (0.70-2.46) uMNewborn (0-30 days old)Both
Normal
details
BloodDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified10.0 (0.0-20.0) uMChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified1.20 +/- 0.11 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.75 (0.0-1.5) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.056 (0.009 - 0.120) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.08 (0.07-0.10) uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.017 +/- 0.005 uMAdult (>18 years old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0-0.15 uMChildren (0 - 10 years old)Both
Normal
    • BC Children's Hos...
details
Cerebrospinal Fluid (CSF)Detected and Quantified0-0.15 uMAdolescent (>11 years old)Both
Normal
    • BC Children's Hos...
details
FecesDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
FecesDetected and Quantified61.166 +/- 74.328 uMNot SpecifiedNot Specified
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothNormal details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
UrineDetected and Quantified0.28 (0.0-0.56) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.03 +/-0.07 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.032 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified< 0.566 umol/mmol creatinineNot AvailableNot AvailableNormal details
UrineDetected and Quantified0 - 25.33 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 13.91 umol/mmol creatinineInfant (1 - 6 months old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 5.09 umol/mmol creatinineInfant (6 months - <1 year old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.64 umol/mmol creatinineChildren (1 - 2 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.64 umol/mmol creatinineChildren (2 - 4 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.64 umol/mmol creatinineChildren (4 - 13 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.64 umol/mmol creatinineAdolescent (13 - 21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0 - 0.64 umol/mmol creatinineAdult (>21 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified0.03 +/- 0.07 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified39.6 uMInfant (0-1 year old)BothInfantile Refsum disease details
BloodDetected and Quantified298.00 (232.00-364.00) uMChildren (1-13 years old)BothPeroxisomal disorders, new type, liver details
BloodDetected and Quantified79.0 (5.0 - 378.0) uMAdult (>18 years old)BothPeroxisomal biogenesis defect details
BloodDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Schizophrenia
details
BloodDetected and Quantified17.95 (5.9-30.00) uMInfant (0-1 year old)BothInfantile Refsum disease details
BloodDetected and Quantified3.0 (0.80-5.3) uMInfant (0-1 year old)BothInfantile Refsum's disease details
Cerebrospinal Fluid (CSF)Detected and Quantified1.8 uMInfant (0-1 year old)BothInfantile Refsum disease details
Cerebrospinal Fluid (CSF)Detected and Quantified0.053 (0.023-0.083) uMAdult (>18 years old)Both
Brain injury
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.19 (0.17-0.21) uMAdult (>18 years old)Not Specified
Malaria
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.38 (0.11-1.26) uMNot SpecifiedNot Specified
Anemia
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.18 (0.14-0.22) uMNot SpecifiedNot SpecifiedMalaria details
Cerebrospinal Fluid (CSF)Detected and Quantified0.17 (0.12-0.23) uMNot SpecifiedNot Specified
Tuberculous meningitis
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.06 (0.02-0.26) uMAdult (>18 years old)Not Specified
Malaria
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.05 (0.02-0.09) uMChildren (1-13 years old)Not Specified
Convulsions
details
Cerebrospinal Fluid (CSF)Detected and Quantified1.715 (0.33 - 3.10) uMAdult (>18 years old)BothPeroxisomal biogenesis defect details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)BothOral cancer details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)FemaleBreast cancer details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Not SpecifiedPancreatic cancer details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Not SpecifiedPeriodontal diseases details
UrineDetected and Quantified2.0 umol/mmol creatinineInfant (0-1 year old)BothInfantile Refsum disease details
UrineDetected and Quantified3.3 (1.5-5.0) umol/mmol creatinineAdult (>18 years old)BothAdrenoleukodystrophy details
UrineDetected and Quantified49.4 (8.4-90.4) umol/mmol creatinineChildren (1-13 years old)BothAdrenoleukodystrophy (ALD) details
UrineDetected and Quantified0.02-228.3 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedZellweger Syndrome details
UrineDetected and Quantified49.00 (8.00-90.00) umol/mmol creatinineChildren (1-13 years old)BothZellweger syndrome details
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Schizophrenia
details
Associated Disorders and Diseases
Disease References
Adrenoleukodystrophy
  1. MetaGene [Link]
Refsum's disease
  1. 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. [2427795 ]
Anemia
  1. 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. [12964115 ]
Traumatic brain injury
  1. 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. [15206793 ]
Convulsion
  1. 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. [12964115 ]
Schizophrenia
  1. 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. [22024767 ]
  2. 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. [24789758 ]
Malaria
  1. 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. [11865422 ]
  2. 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. [12964115 ]
Tuberculous meningitis
  1. 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. [12964115 ]
Peroxisomal biogenesis defect
  1. 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. [12705501 ]
  2. 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. [10509903 ]
  3. 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. [3769211 ]
  4. MetaGene [Link]
Peroxisomal disorders, new type, liver
  1. MetaGene [Link]
Infantile Refsum's disease
  1. MetaGene [Link]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB000545
KNApSAcK IDC00001387
Chemspider ID826
KEGG Compound IDC00408
BioCyc ID5-HYDROXY-PIPECOLATE
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00070
Metagene LinkHMDB00070
METLIN ID50
PubChem Compound849
PDB IDNot Available
ChEBI ID17964
References
Synthesis ReferenceAsher, 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)Download (PDF)
General References
  1. 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. [19212411 ]
  2. 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. [3356409 ]
  3. 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. [6811962 ]
  4. 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. [3769211 ]
  5. 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. [11865422 ]
  6. 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. [1113225 ]
  7. 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. [10894227 ]
  8. Burton BK, Reed SP, Remy WT: Hyperpipecolic acidemia: clinical and biochemical observations in two male siblings. J Pediatr. 1981 Nov;99(5):729-34. [7299546 ]
  9. 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. [15206793 ]
  10. 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. [3116331 ]
  11. 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. [1204235 ]
  12. 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. [12964115 ]
  13. 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. [11270189 ]
  14. 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. [3315316 ]
  15. 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. [12705501 ]
  16. Dancis J, Hutzler J: Comparative rates of metabolism of pipecolic acid in several animal species. Comp Biochem Physiol B. 1982;73(4):1011-2. [6817963 ]
  17. 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. [10509903 ]

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