3 ms·

Do prions typically occur because of a defect in the RNA or are there just certain protein structures that have instability in how they fold?

• type0 6y ago
  because the prions are chaperones, i.e they can fold other proteins
  • jcims 6y ago
    That’s how they turn your brain into Swiss cheese right? I’m just wondering how they are formed, are they misfolded normal proteins or just alien like virii.
    • bbojan 6y ago
      They are misfolded body's normal proteins, with a nasty side effect that they cause the normally folded copies of the same protein to also misfold in the same way. The result is a chain reaction of sorts.
• strbean 6y ago
  In my understanding, a defect in the RNA that affected the protein would necessarily result in a different amino acid sequence. So for prions to form, they would have to be misfolded initially due to chance / environment or re-arranged afterwards through some process. The best known protein to form prions (PrP, Prion Protein) is known to convert from the well-folded form to a misfolded form upon interacting with the misfolded form, which could indicate that spontaneous formation of prions is due to interaction with something after the initial folding. I think this could also just be an indication that PrP just has lots of very close low-energy states, which would mean a higher chance of misfolding initially. - Mostly lay person view (some bioinformatics experience)
  • deleted 6y ago
    [deleted]
• kens 6y ago
  Prions can happen multiple ways. In familial (i.e. hereditary) CJD, a mutation in the PRNP gene (DNA) creates an abnormal misfolded protein, i.e. prion. In sporadic CJD, it's basically bad luck that the protein gets folded wrong. In acquired CJD, exposure to brain tissue with the prion protein causes it. Once there's a prion, it causes more of the protein to misfold, creating more prions and causing the disease.
  • jcims 6y ago
    Dang. Bad luck indeed. Sporadic CJD must feel like you've been struck by lightning.