As our lifetimes get longer and medical science’s diagnoses get more sophisticated, we end up finding new diseases (e.g cancer, Alzheimer’s, diabetes). More often than not, we are unable to treat them because they’re unlike anything we’ve ever encountered. For this reason, I probably won’t become redundant, which is nice. What’s not so nice is that we’ll probably always have the sword of Damocles hanging above our heads, just waiting to be struck down by some intractable lightning bolt. Currently, prion diseases (Bovine Spongiform Encephalopathy, variant Creutzfeldt–Jakob disease, Fatal Familial Insomnia and Kuru) are amongst the scariest.
As we’ve mentioned before, prion diseases are caused by misfolded prion protein. Prion protein is a protein produced naturally by nerve cells with no known function. Certain misfoldings of prion protein change its function: now mutated prion protein can convert normal prion protein into more mutated prion protein. Mutated prion protein builds up in the nerve cells and damages them. Depending on the misfolding, mutated prion protein produces different symptoms, which I’ll explain in the next couple of posts. First off, the incredible story of Kuru.
What really kicked the idea for this post off for me was watching Michael Alpers talk at the RiAus for the opening showing of a documentary on kuru (I highly recommend you watch it on SBSOne – Sunday, December 19 at 8:30PM). Alpers was an Adelaide medical doctor turned researcher when he heard about a “laughing disease” that was affecting the Fore people in the highlands of Papua New Guinea, then an Australian colonial outpost. What was causing this laughing disease? Why was it only the Fore people affected? This piqued his interest so much that he travelled up to PNG and lived amongst the Fore people for more than 40 years to investigate. Continue reading