Tag Archives: Prion

Fatal Familial Insomnia and CJD – Dying to sleep

This post was chosen as an Editor's Selection for ResearchBlogging.org

In mid-1980 Italy, a 52 year old man complains of insomnia, waking easily when he does get sleep, and loss of libido. He knows it’s close to the end for him; he has seen this in his sisters. Within a month, he has fever, starts drooling, cries for no reason. By 2 months, he starts getting out of bed at night and saluting at a hallucinated coronation. He is unable to sleep at all by 3 months.

(Picture taken by Thomas Tu)

Barbiturates and other drugs have no effect. Severe fatigue weighs him down. He would go into fugue states and reenact his dreams. His speech is slurred and incoherent. Eight months in and he has resorted to intermittently spasming and screaming. He cannot recall his dreams. His breathing has become irregular and weak. Nine months after the first symptoms, the man dies from a lung infection. Continue reading

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Kuru – Brain-eating, Nobel-winning and kiddy-fiddling

ResearchBlogging.org

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.

Anton Enus (man with the velvet-smooth voice), Michael Alpers (researcher of kuru), his son Ben Alpers and Robert Bygott (director of the documentary) all have a chat (Picture taken by Thomas Tu)

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

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Mad cow disease – How can something so delicious can be so deadly?

Thomas is off somewhere, I don’t know where, he did tell me but I stopped listening halfway through our conversation and started playing with a slinky. Anyways I’m having to post for him and he decided he wanted to rehash this old article he wrote for On Dit many years ago. Despite the fact I have already written about Mad Cow Disease, Thomas thought his article made some different and interesting points. I just think he’s lazy. Enjoy 🙂

Sexy, sexy proteins

In year 11, my biology teacher (Hi Mr. Everett!) told the class the answer was always proteins; it was what you wrote in exams when you didn’t know the answer. This was because proteins rock because proteins do practically everything in any organism anywhere.

These dogs are composed of and powered by proteins (Rosita Andoreika Baxter Biddophilius Ignatius Theadora a.k.a. Rosie and (Hono)Lulu, Picture taken by Thomas Tu)

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Mad Cow Disease aka Bonkers Beef!

Recently I’ve noticed our posts have strayed from talking about diseases to being about the causative agents themselves. We’ve looked at bacteria and viruses and then shown the diseases they cause. Instead of doing the other way around as was originally intended. Based on this we should re-name the blog ‘Pathogen/Microbe/Thing we want talk about of the Week!’, but it doesn’t have a very good ring to it…

To put things right, and because ‘Mad Cow Disease’ is very cool in its own right, we will return to a disease for our topic this week, rather than another bacteria or virus.

Why would you eat at a café that advertises itself with a mad cow? I wouldn’t go there even if the disease didn’t exist…

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