Development of an antiseizure drug screening platform for Dravet syndrome at the NINDS contract site for the Epilepsy Therapy Screening Program.

ABSTRACT:

Objective

Dravet syndrome (DS) is a rare but catastrophic genetic epilepsy, with 80% of patients carrying a mutation in the SCN1A gene. Currently, no antiseizure drug (ASD) exists that adequately controls seizures. In the clinic, individuals with DS often present first with a febrile seizure and, subsequently, generalized tonic-clonic seizures that can continue throughout life. To facilitate the development of ASDs for DS, the contract site of the National Institute of Neurological Disorders and Stroke (NINDS) Epilepsy Therapy Screening Program (ETSP) has evaluated a mouse model of DS using the conditional knock-in Scn1a^A1783V/WT mouse.

Methods

Survival rates and temperature thresholds for Scn1a^A1783V/WT were determined. Prototype ASDs were administered

SUBMITTER: Pernici CD 

PROVIDER: S-EPMC8360068 | biostudies-literature | 2021 Jul

REPOSITORIES: biostudies-literature