Record Information |
---|
Version | 5.0 |
---|
Status | Expected but not Quantified |
---|
Creation Date | 2024-06-05 20:43:46 UTC |
---|
Update Date | 2024-06-11 21:29:05 UTC |
---|
HMDB ID | HMDB0341532 |
---|
Secondary Accession Numbers | None |
---|
Metabolite Identification |
---|
Common Name | 2S,6S-/2S,6R-oxopropylpiperidine-2-carboxylic acid |
---|
Description | 2S,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 | |
---|
Synonyms | Not Available |
---|
Chemical Formula | C9H15NO3 |
---|
Average Molecular Weight | 185.223 |
---|
Monoisotopic Molecular Weight | 185.105193347 |
---|
IUPAC Name | Not Available |
---|
Traditional Name | Not Available |
---|
CAS Registry Number | Not Available |
---|
SMILES | Not Available |
---|
InChI Identifier | Not Available |
---|
InChI Key | MKEXPIVTMXFKGD-XWIRWTPLNA-N |
---|
Chemical Taxonomy |
---|
Classification | Not classified |
---|
Ontology |
---|
Not Available | Not Available |
---|
Physical Properties |
---|
State | Not Available |
---|
Experimental Molecular Properties | Property | Value | Reference |
---|
Melting Point | Not Available | Not Available | Boiling Point | Not Available | Not Available | Water Solubility | Not Available | Not Available | LogP | Not Available | Not Available |
|
---|
Experimental Chromatographic Properties | Not Available |
---|
Predicted Molecular Properties | Not Available |
---|
Predicted Chromatographic Properties | Predicted Kovats Retention IndicesNot Available |
---|
Spectra |
---|
| |
---|
Biological Properties |
---|
Cellular Locations | Not Available |
---|
Biospecimen Locations | Not Available |
---|
Tissue Locations | Not Available |
---|
Pathways | |
---|
Normal Concentrations |
---|
| Not Available |
---|
Abnormal Concentrations |
---|
| Not Available |
---|
Associated Disorders and Diseases |
---|
Disease References | None |
---|
Associated OMIM IDs | None |
---|
External Links |
---|
DrugBank ID | Not Available |
---|
Phenol Explorer Compound ID | Not Available |
---|
FooDB ID | Not Available |
---|
KNApSAcK ID | Not Available |
---|
Chemspider ID | Not Available |
---|
KEGG Compound ID | Not Available |
---|
BioCyc ID | Not Available |
---|
BiGG ID | Not Available |
---|
Wikipedia Link | Not Available |
---|
METLIN ID | Not Available |
---|
PubChem Compound | Not Available |
---|
PDB ID | Not Available |
---|
ChEBI ID | Not Available |
---|
Food Biomarker Ontology | Not Available |
---|
VMH ID | Not Available |
---|
MarkerDB ID | Not Available |
---|
Good Scents ID | Not Available |
---|
References |
---|
Synthesis Reference | Not Available |
---|
Material Safety Data Sheet (MSDS) | Not Available |
---|
General References | - 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 ]
|
---|