R-2OPP

Biomarker of pyridoxin dependent epilepsy (PDE-ALDH7a1)

Product ID: SV7089
Synonyms: (2S,6R)-6-(2-oxopropyl)piperidine-2-carboxylic acid, 2-OPP, R-2-OPP
Tags: 2-OPP, Biomarker, PDE
<em>R</em>-2OPP
Product Price Estimated Shipping Time Purchase
R-2OPP - 1 mg		 €650.00 1-3 days
R-2OPP - 2 mg		 €950.00 1-3 days
R-2OPP - 5 mg		 €1,600.00 1-3 days
R-2OPP - 10 mg		 €2,200.00 1-3 days
R-2OPP - 50 mg		 €3,200.00 1-3 days
R-2OPP - 100 mg		 €4,500.00 1-3 days

Product information

  • Function

    R-2OPP is a biomarker for pyridoxin dependent epilepsy (PDE).

  • Mode of action

    In pyridoxin dependent epilepsy, a defect in the gene encoding for α-aminoadipic semialdehyde (α-AASA) dehydrogenase (antiquitin), results in the accumulation of α-AASA which is in equilibrium with the reactive Δ1-piperideine-6-carboxylic acid (P6C).1,2 Condensation of P6C with pyridoxal 5′-phosphate (PLP, active form of vitamin B6), results in a deficiency of PLP, thought to be the main pathophysological pathophysiological mechanism of PDE.3

    Reaction of the accumulating P6C with acetoacetate or malonic acid under physiological conditions results in the formation of the biomarkers (2S)-6-(2–oxopropyl)piperidine-2-carboxylic acid (2-OPP) and (2S)-6-(carboxymethyl)piperidine-2-carboxylic acid (CMP).4,5 The formation of another biomarker, 6-oxo-pip, is also formed under a yet unknown mechanism.6

  • Applications

    2-OPP can be applicable as first-tier PDE-ALDH7A1 newborn screening biomarker in dried blood spot (DBS), through a direct-infusion MS method compatible with common newborn screening workflows. Therefore, it can be used as biomarker for treatment monitoring and risk stratification for PDE-ALDH7A1 patients. 4

    A method has been validated that may serve as a routine assay for diagnosis and monitoring of PDE.7

     

  • Chemical Information

    CAS No.: n/a
    SMILES: OC([C@@H]1CCC[C@H](CC(C)=O)N1)=O
    Chemical formula: C9H15NO3
    Molecular weight: 185.22
    Purity: > 95%
    Identity: 1H NMR
    Shipping temperature: 20°C
    Storage temperature: -20°C

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