Text written in Basque and translated automatically by Elia without any subsequent editing. SEE ORIGINAL

[Prions: pathogenic proteins]

Disease of mad cows, fright of sheep or Scrapia. A decade has passed since we discovered these diseases, but they still have no cure.

They are diseases called spongiform encephalopathies, which damage the brain, and the origin of these conditions is in the damaged proteins, the prions. They can be developed not only by animals, but also by humans.

As previously believed, of all mammals, rabbits were among the few that were resistant to prion diseases. But the latest news says the opposite. According to their findings at the CICbioGUNE research center, even rabbits are not safe from these diseases. These are the steps that are being taken in connection with the study of prions.

More than a decade ago, images like this were seen all over the world. Cows and sheep first, and humans later, developed conditions that damaged the brain. They were diseases known as spongiform encephalopathies, caused by prions, which are pathogenic proteins that have the ability to replicate and produce the disease.

The origin of prions remains unknown to researchers.

Steps have been taken at CIC bioGUNE to get to know them better. Being proteins, they know they don't have DNA. That's why it's so hard to destroy. But there are many unanswered questions.

A prion infects a healthy protein with just one touch and turns it into a pathogenic prion, but it is not yet known why this prion is produced, how contact occurs to infect other proteins and what parameters are needed for transformation to occur.

JOKIN CASTILLA; CIC bioGUNE: The protein with the wrong fold, this is the bad protein, is able to bind to other proteins in the brain. Once attached, it causes a structural change in them and turns them into bad proteins. These bad proteins have the potential to infect others. During this multiplication process, the bad protein accumulates and causes the disease. But we still don’t know what mechanism causes the pathology to appear.

Mad cow disease in cows, scrapie in sheep, or Creutzfeldt-Jacobs disease in humans are the most popular prion diseases.

The prion diseases of animals and humans are not always related. It has been shown to be contagious in some cases, such as bovine spongiform encephalopathy. But in others, such as sheep scrapie or chronic deer erosion, there is no contamination. That doesn’t mean it can’t happen over time.

JOKIN CASTILLA; CIC bioGUNE: We all talk about avian or swine flu, and we know that these agents evolve. Prions have the same ability to evolve, and there is the possibility of exposing a prion that is more capable of infecting other animals and/or humans.

Most of the time, prion diseases occur suddenly, but 10% are genetic because they are related to a mutation that will occur in the gene of the protein that will become prion.

It is a case of fatal family insomnia and 40% of the cases in the world occur in the Basque Country. This genetic disease is suddenly manifested and causes permanent insomnia in the patient. The person reaches a state of exhaustion that results in death within a few months.

The discovery of the mechanisms of prions that cause this type of disease is essential to combat these diseases.

JOKIN CASTILLA; CIC bioGUNE: The main lines of research are aimed at finding out the mechanisms by which prions work. Our laboratory investigates precisely what gives a prion the ability to overcome the human limitation, such as why a cow prion is able to infect the human being and a sheep prion is not. We're trying to figure that out. If we knew this, we would be able to block the multiplying power of prions, imitating nature. And we would design drugs for therapy.

The results of these studies would also be useful, for example, for Alzheimer's and Parkinson's diseases, whose mechanisms are similar to those of prion diseases.

JOKIN CASTILLA; CIC bioGUNE: In the case of Alzheimer’s, there is a protein called beta-amyloid, which is also misfolded and has the potential to bind other proteins and misfold them. This is how the Alzheimer's plaques are formed. This is a prionic mechanism.

In the case of Parkinson's, although it is a condition of different origin, it has been observed that, once the basic problems have been solved, patients suffer from a type of late Parkinson's that is related to a protein called alpha-synuclein. This protein behaves similar to prions in its ability to diffuse.

Unfortunately, there is currently no cure for prion diseases. Progress is being made with some antibodies, but it is not very well developed. Researchers estimate that it will be necessary to wait at least five years for effective therapy.