Journal
EPILEPSIA
Volume 52, Issue -, Pages 76-78Publisher
WILEY-BLACKWELL
DOI: 10.1111/j.1528-1167.2011.03008.x
Keywords
Recombinant; Patch-clamp; Dravet syndrome
Categories
Funding
- Biocodex Pharmaceuticals
- NIH-NINDS [RO1-NS045950]
Ask authors/readers for more resources
P>The anticonvulsant stiripentol (Diacomittm) has been shown to have a positive impact on control of seizures for many patients with Dravet syndrome. As with most antiepileptic drugs, stiripentol has multiple mechanisms of action. Its direct anticonvulsant activity is likely due to enhancement of inhibitory, gamma-aminobutyric acid (GABA)ergic neurotransmission. Stiripentol was shown to increase the activity of both neuronal and recombinant GABA(A) receptors at clinically relevant concentrations. At recombinant receptors, stiripentol was found to act through a unique site in a subunit-dependent manner. Positive modulation by stiripentol was most effective at GABA(A) receptors containing an alpha 3 subunit. The expression of the alpha 3 subunit is developmentally regulated, with highest levels in the immature brain. This subunit selectivity may explain the greater clinical efficacy of stiripentol in childhood-onset epilepsies, including Dravet syndrome.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available