Moreles. Photo: Castells

The study clarifies how prion transport occurs -prions are the proteins responsible for ailments such as “mad cow disease.”

The neurodegenerative disease studied by Dr. Moleres, fatal familial insomnia, gravely damages sleep rhythms and causes the death of the patients.

Thus, his research could contribute in the future to the development of therapeutic targets against the third most frequent disease in its category in humans. “As the prions evolve, the damage they cause to the brain increases,” explains Francisco José Moleres.

“In our research we have produced two pioneering animal models which demonstrate that prions propagate in a retrograde direction: from the axons (the long fibers that emerge from the neuron and transmit messages to other cells) to the soma (the most voluminous part of the neuron, where the genetic material is found).”

Research in Spain and USA

As the biologist and biochemist of the University of Navarra explained, this discovery explains “why the encephalic zones first affected in fatal familial insomnia are changed after others which are involved in the generation of sleep. In addition, it clarifies the perturbation of certain brain systems involved in oniric processes.”

The doctoral dissertation was entitled “Distribution and Characterization of the Prionic Protein in the Encephalus of Rodents and in Human Prionic Diseases.” It was performed at the Department of Anatomy of the School of Medicine, under the supervision of José Luis Velayos, and at the National Prion Disease Pathology Surveillance Center of Western Reserve University (USA), under the direction of Pierluigi Gambetti, one of the discoverers of fatal familial insomnia.