The study, published in The Journal of Clinical Investigation (JCI), opens up new horizons for patients with mitochondrial diseases who currently have no therapeutic options when it comes to treating their crises.

“Since the inactivation of STN with deep brain stimulation is already a therapy being used, this option could be considered for patients with Leigh syndrome”, Dr Quintana explains.

At the same time, because they are responsible for inhibiting the STN, their dysfunction led to STN hyperactivity, which triggered seizures.

And the results proved them right! Their analyses showed that inhibitory neurons in the GPe are highly vulnerable to mitochondrial failure, leading to their degeneration.

“We thought that mitochondrial dysfunction in the GABAergic neurons connecting the GPe and the subthalamic nucleus (STN) could be the key”, explains Dr Laura Sánchez-Benito, first author of the study.

They used two different kind of animals: one with whole-body mitochondrial failure, and another where the failure was limited to the inhibitory neurons of a brain region called external globus pallidus (GPe).

Now a team of researchers from @quintanalab.bsky.social @uab.cat @vhir.bsky.social @neuroalc.bsky.social further investigated this type of epilepsy, using mice models of Leigh syndrome, one of the most frequent mitochondrial diseases.

Mitochondrial diseases, rare but severe, affect about one in 4,300 births and often cause untreatable epileptic seizures.

💡 Invertir en Neurociencia es apostar por nuestro futuro.

Lee el manifiesto completo aquí:
👉 www.senc.es/manifiesto-p... (4/4)

#ManifiestoNeurociencia #Ciencia #Innovación #SaludCerebral #SENC

@pgonzalezrodriguez.bsky.social @institutocajal.bsky.social @neuroalc.bsky.social @incuab.bsky.social

And finally, the greatest thanks go to the Sociedad Española de Neurociencias (SENC), which supports us every year, and to whom we are truly grateful.
❤️ It is great to have them on board!

A big thank you to Harvard Bioscience for supporting us as well. With its brands DSI, Panlab and Multi Channel Systems, they bring innovative solutions for telemetry, behavior research and electrophysiology, empowering scientists to advance the frontiers of life sciences.

We’re also thrilled to welcome Werfen! A leader in advanced diagnostics and monitoring solutions, Werfen delivers essential tools that empower research in neuroscience and beyond. Their commitment to innovation drives progress in understanding complex biological processes.

Thank you to Isogen Life Science as well! Specializing in cell biology, molecular biology, and imaging technologies, Isogen provides advanced tools that are essential for accelerating discoveries in neuroscience research.

Izasa Scientific by Palex will be a key partner! They provide cutting-edge equipment and custom solutions for neurobiology, from advanced imaging systems to powerful analytical tools, empowering researchers to push the frontiers of neuroscience.