Characterization of seizure susceptibility in Pcdh19 mice.

ABSTRACT:

Objective

PCDH19-related epilepsy is characterized by a distinctive pattern of X-linked inheritance, where heterozygous females exhibit seizures and hemizygous males are asymptomatic. A cellular interference mechanism resulting from the presence of both wild-type and mutant PCDH19 neurons in heterozygous patients or mosaic carriers of PCDH19 variants has been hypothesized. We aim to investigate seizure susceptibility and progression in the Pchd19 mouse model.

Methods

We assessed seizure susceptibility and progression in the Pcdh19 mouse model using three acute seizure induction paradigms. We first induced focal, clonic seizures using the 6-Hz psychomotor test. Mice were stimulated with increasing current intensities and graded according to a modified Racine scale. We next in

SUBMITTER: Rakotomamonjy J 

PROVIDER: S-EPMC7722218 | biostudies-literature | 2020 Oct

REPOSITORIES: biostudies-literature