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Showing metabocard for 2S,6S-/2S,6R-oxopropylpiperidine-2-carboxylic acid (HMDB0341532)

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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2024-06-05 20:43:46 UTC
Update Date2024-11-19 19:06:39 UTC
HMDB IDHMDB0341532
Secondary Accession NumbersNone
Metabolite Identification
Common Name2S,6S-/2S,6R-oxopropylpiperidine-2-carboxylic acid
Description2S,6S-/2S,6R-OxoPropylPiperidine-2-carboxylic acid, two 2-OPP diastereomers, accumulate in brain tissue and body fluids of patients with Pyridoxin Dependent Epilepsy (#MIM266100; PDE-ALDH7A1) and in Aldh7a1-knockout mice. 2-OPP is a stable biomarker for this disease. It is formed by reaction of P6C with acetoacetate under physiological conditions. 2-OPP induced epilepsy-like behavior in a zebrafish model system. It may contribute to ongoing neurotoxicity, also in treated PDE-ALDH7A1 patients. 2-OPP seems suited as a PDE-biomarker in newborn screening. (PMID: 34138754 )
Structure
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MOLSDFPDBSMILESInChI

MOL for HMDB0341532 (2S,6S-/2S,6R-oxopropylpiperidine-2-carboxylic acid)

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3D MOL for HMDB0341532 (2S,6S-/2S,6R-oxopropylpiperidine-2-carboxylic acid)
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3D SDF for HMDB0341532 (2S,6S-/2S,6R-oxopropylpiperidine-2-carboxylic acid)
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3D-SDF for HMDB0341532 (2S,6S-/2S,6R-oxopropylpiperidine-2-carboxylic acid)
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PDB for HMDB0341532 (2S,6S-/2S,6R-oxopropylpiperidine-2-carboxylic acid)
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3D PDB for HMDB0341532 (2S,6S-/2S,6R-oxopropylpiperidine-2-carboxylic acid)
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SMILES for HMDB0341532 (2S,6S-/2S,6R-oxopropylpiperidine-2-carboxylic acid)
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INCHI for HMDB0341532 (2S,6S-/2S,6R-oxopropylpiperidine-2-carboxylic acid)
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View in JSmolView Stereo Labels
SynonymsNot Available
Chemical FormulaC9H15NO3
Average Molecular Weight185.223
Monoisotopic Molecular Weight185.105193347
IUPAC NameNot Available
Traditional NameNot Available
CAS Registry NumberNot Available
SMILESNot Available
InChI Identifier
InChI=1/C9H15NO3/c1-6(11)4-7-2-3-8(9(12)13)10-5-7/h7-8,10H,2-5H2,1H3,(H,12,13)/t7-,8?/s2
InChI KeyMKEXPIVTMXFKGD-XWIRWTPLNA-N
Chemical Taxonomy
ClassificationNot classified
Ontology
Not AvailableNot Available
Physical Properties
StateNot Available
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular PropertiesNot Available
Predicted Chromatographic Properties
Predicted Kovats Retention Indices
Not Available
Spectra
Biological Properties
Cellular LocationsNot Available
Biospecimen Locations
  • Blood
    • 

  • Cerebrospinal Fluid (CSF)
    • 

  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified<0.34 uMAdult (>18 years old)BothNormal  details
Cerebrospinal Fluid (CSF)Detected and Quantified<0.01 (=LLOQ) uMAdult (>18 years old)BothNormal  details
UrineDetected and Quantified<1.2 umol/mmol creatinineAdult (>18 years old)BothNormal  details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified>1.0 uMAdult (>18 years old)BothPyridoxine-Dependent Epilepsy  details
Cerebrospinal Fluid (CSF)Detected and Quantified>0.11 uMAdult (>18 years old)BothPyridoxine-Dependent Epilepsy  details
UrineDetected and Quantified>0.22 umol/mmol creatinineAdult (>18 years old)BothPyridoxine-Dependent Epilepsy  details
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
External LinksNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Engelke UF, van Outersterp RE, Merx J, van Geenen FA, van Rooij A, Berden G, Huigen MC, Kluijtmans LA, Peters TM, Al-Shekaili HH, Leavitt BR, de Vrieze E, Broekman S, van Wijk E, Tseng LA, Kulkarni P, Rutjes FP, Mecinovic J, Struys EA, Jansen LA, Gospe SM Jr, Mercimek-Andrews S, Hyland K, Willemsen MA, Bok LA, van Karnebeek CD, Wevers RA, Boltje TJ, Oomens J, Martens J, Coene KL: Untargeted metabolomics and infrared ion spectroscopy identify biomarkers for pyridoxine-dependent epilepsy. J Clin Invest. 2021 Aug 2;131(15):e148272. doi: 10.1172/JCI148272. [PubMed:34138754  ]