Researchers from Trinity College Dublin have become the first to describe a model of mitochondrial epilepsy which raises hope for better therapies for patients with this incapacitating condition. Their paper has been published in BRAIN.
Mitochondrial disease is one of the most common forms of genetic diseases, affecting one in 9,000 births in Ireland with debilitating consequences. One quarter of patients with mitochondrial disease have epilepsy which is often severe and resistant towards conventional antiepileptic drugs.
Despite the severity of this epilepsy, up to now there have been no animal models available to provide a mechanistic understanding of the condition.
That is set to change though as researchers at Trinity can now explain the important role that astrocytes play in seizure generation. To date, astrocytes, the characteristic star-shaped glial cells found in the brain and spinal cord, have been seen as ‘supporting-cells’, playing a largely passive assisting role in the brain. This research shows however that they actually play a central role in driving seizure generation in mitochondrial epilepsy.