How brain cells die in prion diseases

The accumulation of protein in axons, as well as other signs of axon swelling and dysfunction, are also early features of other neurodegenerative diseases, including Alzheimer's disease and Parkinson's disease

"We hope that these findings will lead to a better understanding of prions and other neurodegenerative diseases, as well as new strategies to treat them," said Dr.

In their study, the researchers carefully observed the mutant pathogenic copies of the prion disease protein PrP.

The researchers also discovered a key protein complex that is responsible for guiding PrP to axons and causing aggregation associated with axon swelling

Fragile axons

Creutzfeldt-Jakob disease is the most common prion disease in humans, with about one case per million people in the world every year

In this study, Encalada's team used mouse brain cells containing mutant PrP and film technology under the microscope to study the initial accumulation of PrP in axons

The normal function of PrP in neurons has been unclear, but this protein seems to be normally secreted from somatic cells and axons through sac-like containers called vesicles, and is sometimes recovered or degraded in axons.

This movement involves a somewhat complicated vesicle transport system.

Researchers have also discovered a way to combat the formation of endoderm

This result shows a fascinating possibility that prion diseases, as well as many other protein-aggregating diseases in the brain, can be achieved by at least temporarily interrupting the transport of vesicle-encapsulated, aggregated proteins to axons.

Encalada said: "We are very keen to discover molecules that can inhibit this aggregation formation pathway, and to study the role of these inhibitors in animal models of prions and other neurodegenerative diseases

Journal Reference :

1. Romain Chassefeyre, Tai Chaiamarit, Adriaan Verhelle, Sammy Weiser Novak, Leonardo R.