Scientists have built the first model of mitochondrial epilepsy

a recent study, researchers at Trinity College Dublin created a model of mitochondrial epilepsy for the first time, providing a better treatment for patients with this disorder. Their paper is published in brain, a peer-reviewed international journal of neurology.
mitochondrial disease is one of the most common genetic diseases. One in four people with mitochondrial disease has epilepsy, which is usually severe and resistant to conventional anti-epileptic drugs. Nevertheless, there are no animal models that can provide an understanding of the innity of the disease.
so far, asstary glial cells, the characteristic astrological glial cells found in the brain and spinal cord, have been considered "supportive cells" and play a largely passive auxiliary role in the brain. However, the study shows that they actually play an important role in driving mitochondrial seizures.
researchers reconstructed a new brain-slice model by applying astrogenic glial glial cell-specific ocytase inhibitors, fluoro citric acid, along with mitochondrial respiratory inhibitors, fish cane ketones and potassium cyanide.
Then, the team used the model to evaluate the role of astrological glial cells in seizures and demonstrated the involvement of GABA-Glutamate-Glutamine in the cycle, which regulates how chemical emitters are released from neurons and then absorbed by astrological glial cells.
, glutamine appears to be an important intermediate between gabA's ability to suppress the regulation of neurons and astrological cell regions.
, the team found that glutamine synthase deficiency was an important part of the pathogenic process of seizures in brain-slice models and human neuropathological studies.
Responsional research into the importance of research, " said Mark Cunningham, professor of tyrant epilepsy neurophysiology at Ellen Mayston Bates, "We believe this is an important and novel study because it is the first time that a mitochondrial epilepsy model has been produced that captures observed features." The model provides mechanism insights to demonstrate the role of astrological glial cells in this pathological activity. Professor
Cunningham said: "We believe this work is important to provide new ways to provide better treatments for the disease. Future work will further develop this model so that it can be used for new anti-epileptic drugs tailored to patients diagnosed with mitochondrial disease and exhibiting epilepsy symptoms.
source: Researchers describe the first model of mitochondrial epilepsy
Original source: Felix Chan et al, the role of astrocytes in the incapaction generation: insights from from a novel in vitro in thecapration model on mitochondrial dysfunction, Brain (2018). DOI: 10.1093/brain/awy320 (Bio Valley)