Sodium selenate treatment improves symptoms and seizure susceptibility in a malin-deficient mouse model of Lafora disease.

ABSTRACT:

Objective

To search for new therapies aimed at ameliorating the neurologic symptoms and epilepsy developing in patients with Lafora disease.

Methods

Lafora disease is caused by loss-of-function mutations in either the EPM2A or EPM2B genes. Epm2a^-/- and Epm2b^-/- mice display neurologic and behavioral abnormalities similar to those found in patients. Selenium is a potent antioxidant and its deficiency has been related to the development of certain diseases, including epilepsy. In this study, we investigated whether sodium selenate treatment improved the neurologic alterations and the hyperexcitability present in the Epm2b^-/- mouse model.

Results

Sodium selenate ameliorates some of the motor and memory deficits and the sensitivity observe

SUBMITTER: Sanchez-Elexpuru G 

PROVIDER: S-EPMC5495962 | biostudies-literature | 2017 Mar

REPOSITORIES: biostudies-literature