The article, “From Prion Diseases to Prion-Like Propagation Mechanisms of Neurodegenerative Diseases,” was fascinating to read. From what I can gather from the videos and the article, prion is a fatal neurological disease of the brain. Transmission of the disease has been seen genetically, through blood transfusions, or by eating prion-infected animals (scrapie in sheep, bovine in cattle, and chronic wasting in deer). What’s interesting and also unnerving is that it is very difficult to detect prion through blood work. That, along with the fact that prion infections are incurable, shows that there still remains to be a lot of done in this field of study.
It was fascinating to read about the prion cycle and how it infects and multiplies. Form what I learned, PrPsc has the capability to “trigger” the conversion of PrPc (which is normal protein) into PrPsc. Though I wasn’t sure what acted as the trigger, the conversion leads to a misfolding of PrPc, which then leads to the grouping of protofibrils, to amyloid fibrils (fibrils i believe being fibers in the brain) and then a breakdown of the amyloid fibril into potentially new prion agents. When the article goes on to discuss animal testing and mentions that “they do not show all the biochemical and pathological features of prion diseases…,” it reminded me of the limitations animal testing has when it comes to finding results when the disease infects humans (3).
A few questions I had from the readings that someone may help me answer: why was detergent used as a test to see whether the PrPSc would dissolve? What does “residue 90” mean on page 2 when its describing the PrPsc? How does a oligomerization mechanism work/what does it mean? what role does alpha and beta sheets play, and what is recPrP compared to PrPsc? and what does it mean when they are experimenting with prion “in-vitro.”
Also, to end, “The Real Zombie Makers” video begs the question…could prion potentially mutate into the ultimate zombie disease that we’ve all been preparing ourselves for/waiting for?
2 Responses to Week 4: Prions