Excessive Microglia Activation May Underly Seizure Onset in CLN1

June 27, 2022

Article published by Batten Disease News

The number of activated microglia — the brain’s resident immune cells — are significantly increased by the time seizures start to occur in a mouse model of infantile Batten disease, also known as CLN1 disease, a study shows.

Suppressing P2X7R, a receptor protein mainly found at the surface of microglia, significantly reduced the number and total duration of seizures in these mice.

These findings suggest that microglia activation contributes to the development of seizures in infantile Batten, and its suppression may be a new therapeutic avenue for this condition, the researchers noted.

The study, “Seizures in PPT1 Knock-In Mice Are Associated with Inflammatory Activation of Microglia,” was published in the International Journal of Molecular Sciences.