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