Category Archives: Issue 5 November 17

The Mystery of Prions

More Than 30 Years Since Their Discovery, Prions Still fascinate, terrify and mystify us

Figuring out what they were was just the beginning of a field of research into prions and prion diseases that’s still growing

By Kat Eschner 

smithsonian.com
October 6, 2017

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Prions–the name comes from “proteinaceous infectious particle”–were big news in the 1980s, when it became clear that these proteins caused disease. But more than 30 years after they were discovered, we’re still figuring them out.

On October 6th in 1997, American biologist Stanley B. Prusiner received the Nobel Prize in medicine for his discovery of prions, “an entirely new genre of disease-causing agents,” in the words of the Nobel committee. But even though Prusiner’s work started in 1972, by 2017 we still only sort of understand prions.

You’ve probably heard of these infectious proteins in the context of brain diseases like mad cow disease (technical name: bovine spongiform encephalopathy). Humans can also get prion diseases, such as Creutzfeldt-Jacob disease and the rare Kuru, which was transmitted by the Fore people’s custom of eating their deceased as part of funerary rituals. These diseases, which are collectively known as transmissible spongiform encephalopathies, are only the best-understood part of the prion picture.

“Prions are distorted versions of normal proteins found in human and animal brain and other tissues,” explains Colorado State University’s Prion Research Center. “These distorted (‘misfolded’) proteins damage brain cells, leading to fatal dementias akin to human Alzheimer’s and Parkinson’s diseases.”

When normal proteins in your brain–for some reason that’s not fully understood–misfold, “they turn into contagious pathogens that recruit any other prions they come into contact with, grouping together in clumps that damage other cells and eventually cause the brain itself to break down,” writes Fiona MacDonald for ScienceAlert.

“…Technically speaking, proteins shouldn’t be able to infect other proteins–they’re not alive, after all–and scientists have never really been able to explain the behaviour of prions–hence their reputation as the weirdest molecules ever,”  she writes.

Not only are prions not alive (and contain no DNA), they can survive being boiled, being treated with disinfectants, and can still infect other brains years after they were transferred to a scalpel or other tool.

We’re still trying to figure out how normal proteins fold into prions and what causes them to do so, although there have been a number of advances in recent years. Among them, Alzheimer’s and Parkinson’s have recently been linked to prions. Scientists have suggested that these brain diseases are caused by similar protein folding and it has been suggested that they should be called “prionoid” diseases–similiar to TSEs, but not transmissible (that we know of.)

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Product Focus – Tunnel Clip

A new product from Datesand

A simple design made from durable plastic that allows
technicians to suspend a play tunnel from the wire lid of most cages.

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Mini-Bio

julianaJuliana Mckie

Customer Service &  Logistics Advisor

I have been with Datesand a year, and that year has flown by so quickly! I am a Customer Service and Logistics Advisor with a focus on our International Customers. This is the perfect role for me, as I love to travel, and have made friends and contacts from all over the world.

I have been in the UK for 10 years now. I came over the pond to study Archaeology at the University of Leicester in 2007, and met my now husband on the first day of class. We have been married for 4 years. After getting my Masters in Archaeology from the University of Reading, I decided that though I have a passion for history, I also have a passion for administration and logistics, so really focused on following that career path. I cannot imagine doing anything else now and am happy I have been able to follow my dreams, even if they did change along the way.
I grew up in Florida and Kansas, so I try to stay connected to my US roots wherever possible. Which often means inflicting US holidays on my colleagues here at Datesand. Whether it is bringing in a pumpkin pie at Thanksgiving or going out for burgers and barbecue on the 4th of July, I love to share the warmth and enthusiasm of these holidays with others.
I have lived in Manchester for a year and it is such a fantastic city and community. Lately, my husband and I have been exploring the Lake and Peak Districts and we love nothing more than taking long and adventurous walks on the weekends. Next summer, I will be filling in the hundreds of forms to become a dual citizen with both the UK and the US.

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Just for fun:

Which everyday stationery item helped to bring the joint Nobel prize for Physics to Manchester University in 2010?

 

Answer:

Scotch sticky tape

(Two emigré Russian scientists working at the university managed to use sticky tape to peel off thinner and thinner layers of carbon until they got down to a flake just one atom thick. The new material was named graphene and is one of the most unique and potentially useful materials ever developed.)


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Sadly, there aren’t very many of these amazing creatures left on the planet. Can you name the animal and do you know exactly where it lives?

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