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The International School of Advanced Studies (SISSA), in collaboration with the Carlo Besta Neurological Institute in Milan, established ideal lab conditions to create synthetic prions which act like biological ones, in a repetitive manner.
Synthetic prions produced in a series allow precise control over their pathogenic behavior in experiments," explains Giuseppe Legname, coordinator of the study whose lab techniques and results will be published in PLOS Pathogens. Working with 'natural' prions is not always so simple. "They are complex and heterogeneous," says Legname, noting they are often complicated to use. "The synthetic ones we created ourselves, however, are easier to control, homogeneous and structurally defined. And yet they still show the same consequences as biological ones. Our ultimate goal, of course, is to identify mechanisms which can block the pathogenic effect, in order to develop treatments for disease."
In the study, Legname and colleagues synthesized mouse prions, verifying their role in causing illness, which proved comparable to natural prions. "When we 'characterized' them, we also observed that they were very similar to the ones responsible for Mad Cow and Creutzfeldt-Jakob disease, the human form of the illness."
"Naturally, our line of research is already evolving. We will be working with human prions, and we have other projects as well," says Legname. He is referring to the hypothesis which is becoming more and more scientifically sound, that at the base of most neurodegenerative disease there are molecules with mechanisms similar to those of prions. "We are thinking about the molecules responsible for Alzheimers, like amyloid beta, or Parkinsons, or even amyotrophic lateral sclerosis. In these cases as well, having synthetic molecules available could be an important step forward."
Source: International School of Advanced Studies (SISSA)