Stiripentol

Stiripentol is a third-generation antiepileptic compound.¹ It is a positive allosteric modulator of GABA_A receptors, potentiating GABA-mediated activation to a greater extent in receptors expressing α₃ subunits and to a lower extent in those containing β₁ or ε subunits.² It also inhibits GABA reuptake in vitro and increases the release of GABA in neonatal rat hippocampal slices.^1,3 Stiripentol (500 µM) inhibits lactate dehydrogenase (LDH), blocking both lactate-to-pyruvate and pyruvate-to-lactate conversions by human LDH1 and LDH5.⁴ Formulations containing stiripentol have been used in the adjunctive treatment of seizures associated with Dravet syndrome.

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1. Luszczki, J.J. Third-generation antiepileptic drugs: Mechanisms of action, pharmacokinetics and interactions. Pharmacol. Rep. 61(2), 197-216 (2009).

2. Fisher, J.L. The anti-convulsant stiripentol acts directly on the GABA_A receptor as a positive allosteric modulator. Neuropharmacology 56(1), 190-197 (2009).

3. Quilichini, P.P., Chiron, C., Ben-Ari, Y., et al. Stiripentol, a putative antiepileptic drug, enhances the duration of opening of GABA_A-receptor channels. Epilepsia 47(4), 704-716 (2006).

4. Sada, N., Lee, S., Katsu, T., et al. Targeting LDH enzymes with a stiripentol analog to treat epilepsy. Science 347(6228), 1362-1367 (2015).