6-oxo-pip
Biomarker of PDE-ALDH7a1
| Product ID: | SV7072 |
|---|---|
| Synonyms: | (S)-6-oxopiperidine-2-carboxylic acid, 6-oxopipecolate |
| Tags: | Biomarker, PDE |
| Product | Price | Estimated Shipping Time | Purchase |
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€100.00 | 1-3 days | |
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€200.00 | 1-3 days | |
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€300.00 | 1-3 days |
Product information
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Function
6-oxo-pip is a biomarker for pyridoxin dependent epilepsy (PDE). It accumulates in blood, plasma, urine, and cerebral spinal fluid of patients with PDE.
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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 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
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Applications
6-oxo-pip can be used as biomarker for pyridoxin dependent epilepsy.
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Chemical Information
CAS No.: 34622-39-4
SMILES: OC([C@@H](N1)CCCC1=O)=O
Chemical formula: C6H9NO3
Molecular weight: 143.14
Purity: > 95%
Identity: 1H NMR
Shipping temperature: 20°C
Storage temperature: -20°C
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